002 kuliah nutrisi untuk hematopoiesis (3)

8/20/2019 002 Kuliah Nutrisi Untuk Hematopoiesis (3)

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N323 Module B part IN323 Module B part I 11

Hematologic ProblemsHematologic Problems


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N323 Module B part I 2

ANEMIA ANEMIA

1.) Anemia1

.) Anemia is reduction in either RBCs, amount of hemolo!in,is reduction in either RBCs, amount of hemolo!in,or hematocrit "# of pac$ed RBC per deciliter of !lood)or hematocrit "# of pac$ed RBC per deciliter of !lood)

%% Anemia& is a s'mptom of an underl'in disease. Anemia& is a s'mptom of an underl'in disease. Causes and t'pes (ar'Causes and t'pes (ar' 1.)dietar' pro!lems*deficienc' in components necessar'1.)dietar' pro!lems*deficienc' in components necessar'

to ma$e RBC*iron, (itamin B12 "c'anoco!alamin), folicto ma$e RBC*iron, (itamin B12 "c'anoco!alamin), folicacid, or intrinsic factor acid, or intrinsic factor

2.)enetic disorders2.)enetic disorders 3.)!one marro+ disease3.)!one marro+ disease .)e-cessi(e !leedin.)e-cessi(e !leedin .)Immune reactions.)Immune reactions

/.)Chanes in !lood chemistr'/.)Chanes in !lood chemistr' 0.)o-ins in the !lood0.)o-ins in the !lood

** ** Gastrointestinal bleeding common cause of anemia inGastrointestinal bleeding common cause of anemia inadultsadults


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Chronic (s. Acute AnemiaChronic (s. Acute Anemia

Chronic anemiasChronic anemias de(elop raduall',de(elop raduall',more su!tle s'mptoms44 lethar', pallor,more su!tle s'mptoms44 lethar', pallor,

and anore-iaand anore-ia "astritis, hemorrhoids, menstrual"astritis, hemorrhoids, menstrualflo+)flo+)

Acute anemias Acute anemias do not allo+ the !od'do not allo+ the !od'

sufficient time to ma$e ph'sioloicsufficient time to ma$e ph'sioloic

ad5ustments 44 patients s'mptomatic +ithad5ustments 44 patients s'mptomatic +ith

shortness of !reath, e-treme fatiue, andshortness of !reath, e-treme fatiue, andcardiac discomfortcardiac discomfort "trauma, !lood (essel rupture)"trauma, !lood (essel rupture)

http://www.innvista.com/health/ailments/anemias/anemchro.htmhttp://www.innvista.com/health/ailments/anemias/anemchro.htmhttp://www.innvista.com/health/ailments/anemias/anemchro.htm


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N323 Module B part IN323 Module B part I

6ematoloic 7ro!lems6ematoloic 7ro!lems

Anemias Anemias Results inResults in reduction in o-'en transport due toreduction in o-'en transport due to

decrease in hemolo!in production, a decreasedecrease in hemolo!in production, a decrease

in er'throc'tes, or a com!ination of thesein er'throc'tes, or a com!ination of these

factors.factors.

Reduced o-'en leads to less ener' in all cells,Reduced o-'en leads to less ener' in all cells,

reduced cell meta!olism and reproduction.reduced cell meta!olism and reproduction.

Compensation mechanisms include tach'cardiaCompensation mechanisms include tach'cardiaand peripheral (asoconstrictionand peripheral (asoconstriction


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6ematoloic 7ro!lems6ematoloic 7ro!lems

Anemias Anemias 8eneral sins of anemia8eneral sins of anemia fatiue, pallor,fatiue, pallor,

d'spnea, and tach'cardiad'spnea, and tach'cardia

9e(ere anemia ma' lead to anina if9e(ere anemia ma' lead to anina ifo-'en suppl' to the heart is insufficiento-'en suppl' to the heart is insufficient

Chronic se(ere anemia ma' cause C6:Chronic se(ere anemia ma' cause C6:

;ther affects ma' include hair and s$in;ther affects ma' include hair and s$inchaneschanes

Cultural considerationsCultural considerations


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N323 Module B part I /

Key Features of AnemiaKey Features of AnemiaIntegumentary manifestationsInte

gumentary manifestations

Pallor, of ears, nail beds, palmarPallor, of ears, nail beds, palmar

creases, conjunctiva, and aroundcreases, conjunctiva, and aroundmouthmouth

cool to touchcool to touch

intolerance of cold temperaturesintolerance of cold temperatures

Nails become brittle, overtime Nails become brittle, overtime

become concave and fingers are become concave and fingers areclub like in appearance.club like in appearance.

Cardiovascular ManifestationsCardiovascular Manifestations

Tachycardia, murmurs, gallopsTachycardia, murmurs, gallopshen anemia severe orthostatichen anemia severe orthostatic

hypotensionhypotension

!espiratory!

espiratory

ManifestationsManifestations "yspnea on e#ertion"yspnea on e#ertion

"ecreased o#ygen"ecreased o#ygen

saturation levelssaturation levels

Neurologic Manifestations Neurol

ogic Manifestations

Increased somnolenceIncreased somnolence

and fatigueand fatigue

$eadache$eadache


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Diagnostic AssessmentDiagnostic Assessment

Tests of cell number and functionTests of cell number and function::

< Complete blood countComplete blood count

< Reticulocyte countReticulocyte count

< Hemoglobin electrophoresisHemoglobin electrophoresis

< Serum ferritinSerum ferritin

< transferrintransferrin

< total iron-binding capacitytotal iron-binding capacity


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N323 Module B part I =

% LABORATORY PROFILE

Test &ignificance of abnormal finding

Red blood cell count Decreased indicate possibleRed blood cell count Decreased indicate possibleanemia/hemorrhageanemia/hemorrhage

Hemoglobin/Hematocrit Increased indicate possible chronicHemoglobin/Hematocrit Increased indicate possible chronichypoxia, or polycythemia erahypoxia, or polycythemia era

!ean cell hemoglobin "!#$% Increased leels indicate!ean cell hemoglobin "!#$% Increased leels indicate macrocyticmacroc ytic cells,cells,possible anemia& Decreased leelspossible anemia& Decreased leelsindicateindicate microcyticmicroc ytic cells,cells,

possible iron deficiency anemiapossible iron deficiency anemia

Reticulocyte count helpful in determining bone marro' functionReticulocyte count helpful in determining bone marro' function"immature R(#% ))"immature R(#% ))Increased leels indicateIncreased leels indicatechronic blood loss*desireable in anemic clientchronic blood loss*desireable in anemic clientor after hemorrhage&or after hemorrhage&


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N323 Module B part I >

$emoglobin electrophoresis detects abnormal forms of hemoglobin, such$emoglobin electrophoresis detects abnormal forms of hemoglobin, suchasas

hemoglobin & in sickle cell disease,hemoglobin & in sickle cell disease,

Prothrombin time 'IN! assesses e#trinsic clotting cascade,Prothrombin time 'IN! assesses e#trinsic clotting cascade,reflects ho much clottingreflects ho much clotting

factors II, (, (II, ) is functioning.factors II, (, (II, ) is functioning.

Increased Increased *deficient in clotting factor*deficient in clotting factor

cascade.cascade.

Decreased Decreased

*vitamin + e#cess.*vitamin + e#cess.

monitors Coumadin t#- /012 secmonitors Coumadin t#- /012 sec

PTT aPartial thromboplastin time assesses the intrinsic clotting cascade,PTT aPartial thromboplastin time assesses the intrinsic clotting cascade,

factors (III, I), )I, )II.factors (III, I), )I, )II.

Prolonged 'hemophilia orProlonged 'hemophilia ordisseminateddisseminated

intravascular coagulation "IC-.intravascular coagulation "IC-.monitors $eparin-monitors $eparin-

3evel maintained 4./ to ./ times their3evel maintained 4./ to ./ times their

baseline values baseline values


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N323 Module B part I 1?

International normali5ed ration IN!- is theInternational normali5ed ration IN!- is therecommended laboratory measurement system forrecommended laboratory measurement system for

monitoring the effect of oral coagulation therapy.monitoring the effect of oral coagulation therapy.Most patients on oral anticoagulants, theMost patients on oral anticoagulants, thetherapeutic range of the IN! is 01therapeutic range of the IN! is 01

Dehydration: Dehydration: 6hen dehydration is present6hen dehydration is presenthemoconcentration occurs resulting in 7hemoconcentration occurs resulting in 7

increased hemoglobin, hematocrit, serumincreased hemoglobin, hematocrit, serumosmolarity, glucose, protein, blood urea nitrogen,osmolarity, glucose, protein, blood urea nitrogen,and various electrolytes.and various electrolytes.


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1.- $ypoproliferative anemia1.- $ypoproliferative anemia.. $ypoproliferative anemia can$ypoproliferative anemia can be subdivided into three classes based upon the be subdivided into three classes based upon the size of the size of the

RBCs. RBCs. The cells may be larger than normal macrocytic-,The cells may be larger than normal macrocytic-,normal normocytic-, or smaller than normal microcytic-.normal normocytic-, or smaller than normal microcytic-.

% !acrocytic anemia&!acrocytic anemia& !acrocytic anemia can be due to!acrocytic anemia can be due toseeral causes& +he first is a deficiency in itaminseeral causes& +he first is a deficiency in itamin(- or folate, both important ingredients in R(#(- or folate, both important ingredients in R(#

production&production&. !icrocytic anemia&!icrocytic anemia& !icrocytic anemia is due to!icrocytic anemia is due to

abnormalities in the production of the essential R(#abnormalities in the production of the essential R(#protein, hemoglobin& +his is often to due to underlyingprotein, hemoglobin& +his is often to due to underlyingdisease, such as thalassemia, iron deficiency anemiadisease, such as thalassemia, iron deficiency anemia

. /ormocytic anemia /ormocytic anemia & ormocytic anemia may be due to& ormocytic anemia may be due tochronic disease including malnutrition or mixed anemiachronic disease including malnutrition or mixed anemia"combined macrocytic and microcytic anemia%&"combined macrocytic and microcytic anemia%&


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Assessment HematologicAssessment Hematologic Demographic data (age,Demographic data (age,

gendergender

!amily history and genetic ris"!amily history and genetic ris"

#ersonal history (use of ASA,#ersonal history (use of ASA,

$SA%DS, antibiotic use-$SA%DS, antibiotic use-prolonged can lead to boneprolonged can lead to bone

marro& suppressionmarro& suppression

Diet historyDiet history

Socioeconomic statusSocioeconomic status

Current health statusCurrent health status

('ordons Hematologic assessment)('ordons Hematologic assessment)

acti*ity-e+ercise pattern, and nutrition-acti*ity-e+ercise pattern, and nutrition-

metabolic pattern pg .metabolic pattern pg .

S"inS"in Head and nec"Head and nec" RespiratoryRespiratory

Cardio*ascular Cardio*ascular Renal and urinaryRenal and urinary /usculos"eletal/usculos"eletal AbdominalAbdominal

Central ner*ousCentral ner*oussystemsystem #sychosocial#sychosocial


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AnemiaClinical Manifestations

. !ild 0 Hb 1 to 2 g/dl!ild 0 Hb 1 to 2 g/dl

3 !ay exist 'ithout symptoms!ay exist 'ithout symptoms

. !oderate 0 Hb 4 to 1 g/dl!oderate 0 Hb 4 to 1 g/dl

3 Increased cardiopulmonary symptomsIncreased cardiopulmonary symptoms

3 5xperienced at rest or during actiity5xperienced at rest or during actiity

9e(ere @ 6! / dl9e(ere @ 6! / dl

< In(ol(e multiple !od'In(ol(e multiple !od'

s'stemss'stems

InteumentInteument

E'esE'es

MouthMouth

Cardio(ascular Cardio(ascular

7ulmonar'7ulmonar'

NeuroloicNeuroloic

8astrointestinal "8I)8astrointestinal "8I)

Musculos$eletalMusculos$eletal


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ANEMIA9 RE9DIN8 :R;M ECREA9E ANEMIA9 RE9DIN8 :R;M ECREA9E

7R;CI;N ;: RBCs7R;CI;N ;: RBCs

(&(& Iron Deficiency Anemia "R(# small, microcytic%Iron Deficienc y Anemia "R(# small, microcytic% !ild to seere cases!ild to seere cases A sign of underlying problem, important find causeA sign of underlying problem, important find cause 6ccurs all age groups6ccurs all age groups

Insufficient iron impedes synthesis of hemoglobin,Insufficient iron impedes synthesis of hemoglobin,reducing the amounts of oxygen transported inreducing the amounts of oxygen transported inbloodblood

Results inResults in microcytic microcytic "small cell%"small cell% hypochromic hypochromic "less"less

color% erythrocytes due to a lo' concentration ofcolor% erythrocytes due to a lo' concentration ofhemoglobin in each cell&hemoglobin in each cell&


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Iron4eficienc' AnemiaIron4eficienc' Anemia

;ne of the most common chronic;ne of the most common chronic

hematoloic disordershematoloic disorders

Iron is present in all RBCs as heme inIron is present in all RBCs as heme in

hemolo!in and in a stored formhemolo!in and in a stored form

6eme accounts for t+o thirds of the6eme accounts for t+o thirds of the

!od'Fs iron!od'Fs iron


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N323 Module B part I 1/

Iron eficienc' Anemia "RBC small, microc'tic)Iron eficienc' Anemia "RBC small, microc'tic)

#auses#auses* depleted iron stores "decreased ferritin%,* depleted iron stores "decreased ferritin%,

results in a decreased supply of iron for theresults in a decreased supply of iron for themanufacture of hemoglobin in R(#smanufacture of hemoglobin in R(#s Iron stores -/7 hemoglobin, /7Iron stores -/7 hemoglobin, /7rdrd in the bone marro',in the bone marro',

spleen, lier, and musclespleen, lier, and muscle !any causes8!any causes8

9ac: of dietary inta:e of iron containing foods9ac: of dietary inta:e of iron containing foods"adolescent gro'th spurt, pregnancy need higher"adolescent gro'th spurt, pregnancy need higheramount of iron%amount of iron%

;lo', chronic blood loss from bleeding ulcer,;lo', chronic blood loss from bleeding ulcer,

hemorrhoids, cancer, or excessie menstrual flo'hemorrhoids, cancer, or excessie menstrual flo' syndromes of


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Signs and Symptoms:Signs and Symptoms:

Mild anemia freGuentl' as'mptomaticMild anemia freGuentl' as'mptomatic 7allor s$in and mucous mem!ranes7allor s$in and mucous mem!ranes

:atiue, lethar':atiue, lethar'

Cold intolerance "caused !' decreased cellCold intolerance "caused !' decreased cellmeta!olism)meta!olism)

Irrita!ilit' " CN9 h'po-ia)Irrita!ilit' " CN9 h'po-ia)

eenerati(e chanes includin !rittle hair, spooneenerati(e chanes includin !rittle hair, spoonshaped "conca(e) rided nails,shaped "conca(e) rided nails,

:emales menstrual irreularities:emales menstrual irreularities

9e(ere anemia tach'cardia, palpitations, d'spnea,9e(ere anemia tach'cardia, palpitations, d'spnea,s'ncope, as +ell as dela'ed healin.s'ncope, as +ell as dela'ed healin.


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N323 Module B part I 1=


Diagnostic +ests8Diagnostic +ests8

Hemoglobin, hematocrit "lo'%Hemoglobin, hematocrit "lo'%!ean corpuscular olume "!#$%, mean!ean corpuscular olume "!#$%, mean

corpuscular hemoglobin "!#H%corpuscular hemoglobin "!#H%

;erum ferritin less than- g/9;erum ferritin less than- g/9;erum iron;erum iron5rythrocytes appear hypochromic and5rythrocytes appear hypochromic and

microcytic on microscopic examinationmicrocytic on microscopic examination


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N323 Module B part I 1>


!anagement*!anagement*

Find underlying causeFind underlying cause increase oral inta:e of iron, iron supplementsincrease oral inta:e of iron, iron supplements

"ta:e '/food to reduce gastric irritation and"ta:e '/food to reduce gastric irritation andnausea%nausea%

6ral6ral treatment in 2 'ee:s raises Hgb abouttreatment in 2 'ee:s raises Hgb about-/g/dl-/g/dl

9i=uid form9i=uid form of iron stains teeth and denturesof iron stains teeth and dentures*use stra'*use stra'

I!I!*use >?trac: method,*use >?trac: method, (entro4luteal(entro4lutealin5ectionin5ection do not massage site do not massage site


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Drug Therapy Drug Therapy . 6ral iron6ral iron3 $itamin # aids in iron absorption$itamin # aids in iron absorption3 Factors to consider "cont@d%Factors to consider "cont@d%

. (est absorbed as ferrous sulfate in an acidic(est absorbed as ferrous sulfate in an acidicenironmentenironment

. 9i=uid iron should be diluted and ingested9i=uid iron should be diluted and ingestedthrough a stra' to preent staining of teeththrough a stra' to preent staining of teeth

. ;ide effects;ide effects

3 Heartburn, constipation, blac: stools,Heartburn, constipation, blac: stools,diarrheadiarrhea


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Nursing Management Nursing Management . At?ris: groupsAt?ris: groups3 Premenopausal 'omenPremenopausal 'omen

3 Pregnant 'omenPregnant 'omen

3 Persons from lo' socioeconomicPersons from lo' socioeconomicbac:groundsbac:grounds

3 6lder adults6lder adults

3 Indiiduals experiencing blood lossIndiiduals experiencing blood loss


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Nursing Management Nursing Management . Diet teachingDiet teaching. ;upplemental iron;upplemental iron

. Discuss diagnostic studiesDiscuss diagnostic studies. 5mphasie compliance5mphasie compliance

. Iron therapy for - to 7 months afterIron therapy for - to 7 months afterthe hemoglobin leels return to normalthe hemoglobin leels return to normal


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Common !ood sources of %ron, 0itaminCommon !ood sources of %ron, 0itamin

123, and !olic Acid123, and !olic Acid

Iron $itamin (-Iron $itamin (-

lier , organ meats lier, organ meats, nutslier , organ meats lier, organ meats, nuts

'hole 'heat breads/cereals dried beans, green leafy'hole 'heat breads/cereals dried beans, green leafy

egeseges 9eafy green leafy eges, carrots citrus fruit, bre'ers9eafy green leafy eges, carrots citrus fruit, bre'ers

yeast yeast

5gg yol:s, raisins5gg yol:s, raisins

Folic AcidFolic Acid

9ier, organ meats, eggs, cabbage9ier, organ meats, eggs, cabbage

(rocolli, brussel sprouts(rocolli, brussel sprouts


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Mealo!lastic AnemiasMealo!lastic Anemias

8roup of ; caused !' NA s'nthesis and8roup of ; caused !' NA s'nthesis and

characteriHed !' lare RBCs "a!normal @characteriHed !' lare RBCs "a!normal @

hemol'sis)hemol'sis)

CommonCommon Vitamin B12 CobalaminVitamin B12 Cobalamin deficienc'deficienc'anemiaanemia

ernicious anemiaernicious anemia < lac$ of secretion of intrinsic < lac$ of secretion of intrinsic

factor "needs acidic en(ironment) in 8I tract +hichfactor "needs acidic en(ironment) in 8I tract +hich

is necessar' for a!sorption of itamin B12is necessar' for a!sorption of itamin B12 !olic acid anemia!olic acid anemia

;ther causes certain drus;ther causes certain drus


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0itamin 123 Deficiency Anemia0itamin 123 Deficiency Anemia (Cobalamin(Cobalamin 44Pernicious anemiaPernicious anemia (megaloblastic, macrocytic (megaloblastic, macrocytic

. #haracteried by ery large nuclueated,#haracteried by ery large nuclueated,immature erythrocyteimmature erythrocyte

. $itamin (- is re=uired for R(# maturation$itamin (- is re=uired for R(# maturation

. #auses8#auses8 deficiency in (- "cyanocobalamin%deficiency in (- "cyanocobalamin%

results in inhibiting folic acid acid transport intoresults in inhibiting folic acid acid transport intothe cell 0R(# maturity does not can occur& "allthe cell 0R(# maturity does not can occur& "allcell diision re=uires ade=uate amount of foliccell diision re=uires ade=uate amount of folicacid to ma:e DA%acid to ma:e DA%

0it i 1230it i 123 D fi iD fi i


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N323 Module B part IN323 Module B part I 2/2/

0itamin 1230itamin 123 (Cobalamin(Cobalamin DeficiencyDeficiency

Anemia 4Anemia 4 Pernicious anemiaPernicious anemia

(megaloblastic, macrocytic(megaloblastic, macrocytic Results from poor inta$e of B12 related toResults from poor inta$e of B12 related to(eetarian diets, or diets lac$in in dair'(eetarian diets, or diets lac$in in dair'

products,products,

"# "# poor a!sorption of B12 in conditionspoor a!sorption of B12 in conditions

such as small !o+el resection, tape+orm,such as small !o+el resection, tape+orm,

or o(erro+th of intestinal !acteriaor o(erro+th of intestinal !acteria

itamin B12 eficienc' Anemia Jam n e c enc' nem a


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itamin B12 eficienc' Anemia Jam n e c enc' nem a7ernicious anemia "mealo!lastic,7ernicious anemia "mealo!lastic,

macroc'tic)macroc'tic)

Ke' :eatures of itamin B12 AnemiaKe' :eatures of itamin B12 Anemia se(ere pallor se(ere pallor

sliht 5aundicesliht 5aundice

9mooth, shin', !eef' tonue9mooth, shin', !eef' tonue

+eiht loss+eiht loss

paresthesia paresthesia of hands and feet "num!ness andof hands and feet "num!ness andtinlin), difficult' +ait related lac$ of B12tinlin), difficult' +ait related lac$ of B12

needed for normal ner(e function "impairedneeded for normal ner(e function "impairedconduction of ner(e impulses@dem'elination)conduction of ner(e impulses@dem'elination)peripheral ner(es and e(entuall' spinal cord)peripheral ner(es and e(entuall' spinal cord)


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N323 Module B part IN323 Module B part I 2=2=

Vitamin B12 Deficiency Anemia &Vitamin B12 Deficiency Anemia & PerniciousPerniciousanemia anemia ) (megaloblastic, macrocytic)) (megaloblastic, macrocytic)

. Diagnostic tests8Diagnostic tests8

. 5rythrocytes appear macrocytic or megaloblastic5rythrocytes appear macrocytic or megaloblastic

. ;erum itamin (- lo';erum itamin (- lo'

. ;hilling test;hilling test is used to measure absorption of itaminis used to measure absorption of itamin

(- after oral administration&(- after oral administration&. +reatment8+reatment8 prophylaxis oral supplements forprophylaxis oral supplements forpregnant 'omen and egetarians&pregnant 'omen and egetarians&

. $itamin (- inBection$itamin (- inBection daily C- 'ee:s daily C- 'ee:s initially, theninitially, then

'ee:ly until hemoglobin normal, then monthly for'ee:ly until hemoglobin normal, then monthly formaintenance for lifemaintenance for life


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N323 Module B part IN323 Module B part I 2>2>

!olic acid deficiency (also cause!olic acid deficiency (also causemegaloblastic anemiamegaloblastic anemia

!e8uired for "N9 synthesis 3'T !:C formation ;!e8uired for "N9 synthesis 3'T !:C formation ;

maturationmaturationManifestations7Manifestations7 similar to (itamin :4 anemia,similar to (itamin :4 anemia, except except

nervous system function remains normal. "iseasenervous system function remains normal. "iseasedevelops sloly.develops sloly.

Causes7Causes7 common include poor nutrition,r't chroniccommon include poor nutrition,r't chronicalcoholism-, malabsorption problems Crohns disease-,alcoholism-, malabsorption problems Crohns disease-,and drugs oral contraceptives, anticonvulsants-, $"and drugs oral contraceptives, anticonvulsants-, $"

Collaborative management7Collaborative management7

Identif' hih ris$ roups "elderl', de!ilitated, alcoholic,Identif' hih ris$ roups "elderl', de!ilitated, alcoholic,those suscepti!le to malnutrition, and those +ho reGuirethose suscepti!le to malnutrition, and those +ho reGuiremore "prenanc')more "prenanc')

9cheduled folic acid replacement therap'9cheduled folic acid replacement therap'


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halassemia "Coole's anemia)

$tiology An autosomal recessi(e enetic disorder of inadeGuateproduction of normal hemolo!in

Normal hemolo!in, also called hemolo!in A, has four

protein chains*t+o alpha lo!in and t+o beta lo!in.

In halassemia some of these proteins are missin< D

a!normal hemolo!in

< Common in ethnic roups near the Mediterranean

9ea and eGuatorial reions of Asia and Africa


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halassemia

$tiology 6emol'sis also occurs 7ro!lem +ith lo!ulin protein


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halassemia

Clinical Manifestations halassemia minor < As'mptomatic freGuentl'

< Moderate anemia 9plenomeal'

Mild 5aundice halassemia ma5or

< Dife4threatenin

< 7h'sical and mentalro+th often retarded

< 7ale

< 9'mptoms de(elop inchildhood

halassemia ma5or

< 9plenomeal'

< 6epatomeal'

< Laundice from

hemol'sis < Chronic !one marro+

h'perplasia E-pansion of !one

marro+ space


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halassemia

Collaborati%e Care No specific dru or

diet is effecti(e intreatin thalassemia

halassemia minor < Bod' adapts todecreased 6!

halassemia ma5or

< Blood transfusions+ith I defero-amine"!inds +ith iron, rids throuh$idne's, , hematochromotosis)

7re(ention

8enetic counselin

+ith families +ith

$no+n histor' ofhalassemia

7renatal chec$up


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'lucose-5-#hosphate Dehydrogenase ('5#D'lucose-5-#hosphate Dehydrogenase ('5#D

Deficiency AnemiaDeficiency Anemia

Most common t'pe of conenital hemol'tic anemiaMost common t'pe of conenital hemol'tic anemia

+here this+here this en&ymeen&yme is lac$in in the RBCis lac$in in the RBC

EnH'me lac$in in the critical step of RBC ener'EnH'me lac$in in the critical step of RBC ener'

productionproduction

hen e-posed to certain drus or to-ins the ain RBChen e-posed to certain drus or to-ins the ain RBC

easil' !rea$easil' !rea$

Client usuall' as'mptomatic until anemia or se(ereClient usuall' as'mptomatic until anemia or se(ere

infection de(elopsinfection de(elops Acute phase anemia and 5aundice Acute phase anemia and 5aundice

6emol'tic reaction is limited !ecause onl' older RBCs6emol'tic reaction is limited !ecause onl' older RBCs

containin less 8/7, are destro'edcontainin less 8/7, are destro'ed


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'lucose-5-#hosphate Dehydrogenase ('5#D'lucose-5-#hosphate Dehydrogenase ('5#D

Deficiency AnemiaDeficiency Anemia

Collaborati*e management:Collaborati*e management: Hydration during episode of hemolysis toHydration during episode of hemolysis to

pre*ent acute tubular necrosispre*ent acute tubular necrosis

%dentify and remo*e to+ins or drug%dentify and remo*e to+ins or drug

Screening for this deficiency necessary beforeScreening for this deficiency necessary before

donating blooddonating blood

6smotic diuretic such as mannitol (6smitrol6smotic diuretic such as mannitol (6smitrol

1lood transfusion to correct anemia1lood transfusion to correct anemia


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Aplastic Anemia (macrocytic)

#ause8 deficiency of circulation R(# due to failure of bonemarro' to produce these R(# cells, may occur alone or 'ith

Leuo!enia ("ecrease" #BC) an" t$rombocyto!enia ("ecrease" !latelets)% #$en ALL t$ree

occur toget$er it is calle" &Pancyto!enia' #ause8

&% #ongenital in origin 3 chromosomal abnormality 2%) Ac=uired??9ong term exposure to toxic agents, ioniingradiation or infection, "iral, bacterial%, medications,antiseiure, antimicrobials% may cause Aplastic anemia&

7&% 1E of ac=uired is idiopathic "un:no'n cause%


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&igns and &ymptoms7


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% 9plastic 9nemia macrocytic- Diagnosis8 definitie bone marro' aspiration red

bone marro' is replaced by fatty red bone marro' +reatment8 blood transfusions, immunosuppressie

therapy "antilymphocyte globulin "A9


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Anemias of Chronic isease

Chronic inflammator'

Autoimmune

Infectious

Malinant diseases

E-amples

Renal disease "decreased ere'thropoietin)

M'elosuppression Medication*chemotherap'

Radiation


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N323 Module B part IN323 Module B part I ??

Acute Blood Doss Acute Blood Dossandand

Chronic Blood DossChronic Blood Doss


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Acute Blood Doss Acute Blood Doss

2?# (olume loss ma' see sins or s'mptoms +ith increased2?# (olume loss ma' see sins or s'mptoms +ith increased

acti(it', sliht postural h'potensionacti(it', sliht postural h'potension

Result of sudden hemorrhaeResult of sudden hemorrhae

< raumarauma

< Complications of surer'Complications of surer' < isruption (ascular interit'isruption (ascular interit'

ConcernsConcerns

< 6'po(olemic shoc$6'po(olemic shoc$

< Reduced plasmaReduced plasma

(olume(olume

iminished ;iminished ;22

!ecause fe+er RBCs!ecause fe+er RBCs

a(aila!lea(aila!le


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Clinical ManifestationsClinical Manifestations

CauseCause

< Bod'Fs attempt to maintain an adeGuate !lood (olumeBod'Fs attempt to maintain an adeGuate !lood (olume

and ;and ;22

7ain7ain < Internal hemorrhaeInternal hemorrhae

issue distention, oran displacement, ner(e compressionissue distention, oran displacement, ner(e compression 7ain "contOd)7ain "contOd)

< Retroperitoneal !leedinRetroperitoneal !leedin Num!nessNum!ness

7ain in the lo+er e-tremities7ain in the lo+er e-tremities

9hoc$ is the ma5or complication9hoc$ is the ma5or complication


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Diagnostic 'tudiesDiagnostic 'tudies

Da!orator' data does not adeGuatel'Da!orator' data does not adeGuatel'

assess RBC pro!lems for 2 to 3 da'sassess RBC pro!lems for 2 to 3 da's

Collaborati%e CareCollaborati%e Care

Replacin !lood (olume to pre(ent shoc$Replacin !lood (olume to pre(ent shoc$

Identif'in the source of the hemorrhaeIdentif'in the source of the hemorrhae

9toppin !lood loss9toppin !lood loss

Correctin RBC lossCorrectin RBC loss


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Nursing Management Nursing Management

Ma' !e impossi!le to pre(ent if caused !'Ma' !e impossi!le to pre(ent if caused !'

traumatrauma

7ostoperati(e patients7ostoperati(e patients

< Monitor !lood lossMonitor !lood loss

No need for lon4term treatmentNo need for lon4term treatment


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Chronic Blood DossChronic Blood Doss

Reduced iron storesReduced iron stores

< Bleedin ulcer Bleedin ulcer

< 6emorrhoids6emorrhoids

< Menstrual and postmenopausal !lood lossMenstrual and postmenopausal !lood loss ManaementManaement

< Identif' sourceIdentif' source

< 9top !leedin9top !leedin

< 7ossi!le use of supplemental iron7ossi!le use of supplemental iron


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6emol'tic Anemia

estruction or hemol'sisof RBCs at a rate thate-ceeds production

hird ma5or cause of

anemia Intrinsic hemol'tic

anemia


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6emol'tic Anemia

Laundice

< estro'ed RBCs

cause increased

!iliru!in

Enlared spleen and

li(er

< 6'peracti(e +ith

macrophaephaoc'tosis of the

defecti(e RBCs

Accumulation of

hemolo!in

molecules can

o!struct renal tu!ules < u!ular necrosis


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2.) 6emol'tic Anemias2.) 6emol'tic Anemias

resultin fromresultin from

increased destructionincreased destruction"hemol'sis) of RBCs"hemol'sis) of RBCs

a.) 9ic$le cell diseasea.) 9ic$le cell disease

**

!.)!.) 8lucose4/48lucose4/47hosphate7hosphate

eh'droenaseeh'droenase

eficienc' anemiaeficienc' anemia

c&%c&% +hrombotic+hrombotic+hrombocytopenia+hrombocytopenia

Purpura "++P%Purpura "++P% is ais arare autoimmunerare autoimmunereaction in bloodreaction in bloodessels disorder inessels disorder in

'hich'hich platelets platelets clumpclumptogether abnormally intogether abnormally inthe capillaries and fe'the capillaries and fe'remain in circulation&remain in circulation&

;i :l # ll Di " i lti f;i :l # ll Di

" i lti f


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;ic:le #ell Disease "can cause anemia resulting from;ic:le #ell Disease "can cause anemia resulting fromincreased destruction of R(#s%increased destruction of R(#s%

&% #ause&% #ause* genetic defect causing a formation of abnormal* genetic defect causing a formation of abnormalhemoglobin chains "Hb;% resulting in chronic anemia, pain,hemoglobin chains "Hb;% resulting in chronic anemia, pain,disability, organ damage, increased ris: for infection, anddisability, organ damage, increased ris: for infection, andearly deathearly death

► ;ic:le cell disease occurs in in 71?11 African Americans;ic:le cell disease occurs in in 71?11 African Americans ► ;ic:le cell disease state s& sic:le cell trait, ariation of;ic:le cell disease state s& sic:le cell trait, ariation of

seerityseerity► ;ic:le cell trait ;ic:le cell trait 3less than 21E of total hemoglobin Hb;3less than 21E of total hemoglobin Hb;► ;ic:le cell disease ;ic:le cell disease 3 G1?11E of total hemoglobin contains3 G1?11E of total hemoglobin contains

may hae abnormality of hemoglobin ; "Hb;%may hae abnormality of hemoglobin ; "Hb;%


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N323 Module B part I ?

-&% Insufficient oxygen-&% Insufficient oxygen0 cells to assume a0 cells to assume a

sic:le shape,sic:le shape, cells become rigid andcells become rigid andclumped together,clumped together,

obstructing small bloodobstructing small bloodessels or the capillaryessels or the capillary

blood flo', cause enousblood flo', cause enousstasis, anemia, pain,stasis, anemia, pain,enlarged spleenenlarged spleen

other manifestations inother manifestations inthe respiratory,the respiratory,

genitourinary,genitourinary,cardioascular,cardioascular,musculos:eletal andmusculos:eletal andintegumentary systems&integumentary systems&

6bstruction of the small6bstruction of the smallblood essels 0 repeatedblood essels 0 repeatedmultiple infarctions ormultiple infarctions or

areas of tissue necrosisareas of tissue necrosisthroughout throughout the bodythe body Deoxygenation ofDeoxygenation of

hemoglobin in peripheralhemoglobin in peripheralcirculation 0 sic:le cellcirculation 0 sic:le cell

crisiscrisis


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Clinical /anifestationsClinical /anifestations

Cardio*ascular changesCardio*ascular changes

S"in changesS"in changes

Abdominal changesAbdominal changes

/usculos"eletal changes/usculos"eletal changes Central ner*ous system changesCentral ner*ous system changes


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. DiagnosisDiagnosis

. Hemoglobin ; "Hb;% electrophoresisHemoglobin ; "Hb;% electrophoresis

. Diagnosis confirmed by the presence ofDiagnosis confirmed by the presence ofsic:led cells in peripheral bloodsic:led cells in peripheral blood

. Hematocrit lo'Hematocrit lo'

. Reticulocyte count high "chronic anemia%Reticulocyte count high "chronic anemia%

. !ean corposcular hemoglobin!ean corposcular hemoglobinconcentration "!#H#% highconcentration "!#H#% high

. bilirubin leels are highbilirubin leels are high

. J(#s eleated "due to chronicJ(#s eleated "due to chronicinflammation and hypoxia%inflammation and hypoxia%


53/120


54/120

N323 Module B part I

Care of the Client inCare of the Client in

9ic$le Cell Crisis9ic$le Cell Crisis

Administer o-'en Administer o-'en

#ain#ain

< Drug therapy: 7Drug therapy: 7

hours of intra*enoushours of intra*enous

analgesicsanalgesics

< "opiods*Morphine or"opiods*Morphine or

ilaudid) I or 7CAilaudid) I or 7CA

6'drate +ith I:,6'drate +ith I:,

encourae 7; fluidsencourae 7; fluidslitersda'litersda'

Administer !lood Administer !lood

transfusion if reGuiredtransfusion if reGuired Remo(e constricti(eRemo(e constricti(e

clothinclothin

Encourae client to $eepEncourae client to $eep

e-tremities e-tended toe-tremities e-tended topromote (enous returnpromote (enous return

Chec$ circulation ofChec$ circulation of

e-tremitiese-tremities

Complementary andComplementary and

alternati*e therapiesalternati*e therapies

# t ti l f / lti l 6# t ti l f / lti l 6


55/120

N323 Module B part I

#otential for /ultiple 6rgan#otential for /ultiple 6rganDysfunctionDysfunction

%nter*entions include:%nter*entions include: -- pain managementpain management < Hydration %0, #6Hydration %0, #6

<6+ygen therapy6+ygen therapy < Transfusion therapyTransfusion therapy

< Hydro+yurea (Dro+ia-Hydro+yurea (Dro+ia-(stimulate Hb!, fetal hemoglobin(stimulate Hb!, fetal hemoglobinproduction, reducing HbS, 8T use S9 bone marro& suppression, causesproduction, reducing HbS, 8T use S9 bone marro& suppression, causesbirth defects)females should use 3 methods of birth controlbirth defects)females should use 3 methods of birth control

< Complementary and Alternati*eComplementary and Alternati*eTherapiesTherapies

(T$S, acupuncture(T$S, acupuncture

AcGuired 6emol'tic Anemia


56/120



hree e-trinsic cateories

1. 7h'sical factors

Physical destruction of R(#sresults from extremeforce on the cells

3 Hemodialysis,extracorporealcirculation,prosthetic heartales, angiopathicdisease "any disease ofessels%

2. Immune reactions


57/120



hree e-trinsic cateories

3. Infectious aents and to-ins

< :oster hemol'sis in four +a's

< In(adin RBCs and destro'in contents < Releasin hemol'sis su!stances

< 8eneratin an antien


58/120

N323 Module B part IN323 Module B part I ==

HemochromatosisHemochromatosis

7rimar' hemochromatosis is aninherited disorder characteriHed !'

e-cessi(e iron accumulation due to

increa-ed intestinal iron a!sorption

causin tissue damae.

9'mptoms do not de(elop until oran

damae, often irre(ersi!le, de(elops.


59/120

N323 Module B part IN323 Module B part I >>

. ;ymptoms include fatigue,

hepatomegaly, brone s:inpigmentation, loss of libido,arthalgias, and manifestations of

cirrhosis, diabetes, orcardiomyopathy&

. Diagnosis is based on serum iron

studies and gene assay&. +reatment serial phlebotomies

AnemiaAnemia


60/120

N323 Module B part IN323 Module B part I /?/?

Anemia Anemia

Nursing (mplementationNursing (mplementation ietar' and lifest'le chanesietar' and lifest'le chanes

Blood or !lood product transfusionsBlood or !lood product transfusions

ru therap'ru therap'

;-'en therap';-'en therap' 7atient teachin7atient teachin

< Nutrition inta$eNutrition inta$e

< Compliance +ith dru therap'Compliance +ith dru therap'

GerontologicGerontologic

ConsiderationsConsiderations

Common in older adultsCommon in older adults

< Chronic diseaseChronic disease

< Nutritional deficienciesNutritional deficiencies

9ins and s'mptoms ma'9ins and s'mptoms ma'

o unreconiHed oro unreconiHed or

mista$en for normal ainmista$en for normal ain

chaneschanes


61/120

7ol'c'themia 7roduction and

presence of increasedRBCs

2 t'pes

7rimar' pol'c'themia @7ol'c'themia era

9econdar' 7ol'c'themia @a.) h'po-ia dri(en @ hihaltitude, cardiopulmonar'

disease, defection ;2transport

!.)6'po-ia independent@renal c'sts or tumors

. Polcycyt$emiaPolcycyt$emiaVera.(PV) is a rareVera.(PV) is a rare"isease /it$ a"isease /it$ asustaine" increase insustaine" increase inbloo" cells (!rimarilybloo" cells (!rimarily

re" bloo" cells)re" bloo" cells)!ro"uce" by t$e bone!ro"uce" by t$e bonemarro/marro/

. #it$ unno/n origin,#it$ unno/n origin,c$romosomal "efect inc$romosomal "efect in

!luri!otent stem cells!luri!otent stem cells


62/120

N323 Module B part I /2

. t is a cancer of t$e 0BCs /it$ t is a cancer of t$e 0BCs /it$ maor $allmars*maor $allmars*

. 1%) Massie !ro"uction of1%) Massie !ro"uction of re" bloo"re" bloo"cells cells

. 2%) 3-cessie2%) 3-cessie leuocyte leuocyte !ro"uction!ro"uction

. %) 3-cessie !ro"uction%) 3-cessie !ro"uction of !latelets of !latelets

Hgb leels to G g/dlHgb leels to G g/dlHct of E or Hct of E or

R(# count of 4 mil/mm7R(# count of 4 mil/mm7

#olycythemia 0era#olycythemia 0era


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N323 Module B part I /3

#olycythemia 0era#olycythemia 0eraDue to hyperiscousDue to hyperiscous

"thic:er than normal"thic:er than normal

blood% the follo'ing mayblood% the follo'ing mayoccur8occur8

. Key features8Key features8

. #lient@s facial s:in and#lient@s facial s:in and

mucous membranes haemucous membranes haea dar:, flusheda dar:, flushed"plethoric% appearance"plethoric% appearance

. Distention of superficialDistention of superficialeinseins

. Jeight lossJeight loss

. Intense itchingIntense itching

. HypertensionHypertension

. Fatigue, enlargedFatigue, enlargedhemorrhoidshemorrhoids

. ;'ollen painful Boints;'ollen painful Boints

. 5nlarged firm spleen5nlarged firm spleen

. Infarctions of the heartInfarctions of the heart"chest pain, heart"chest pain, heartfailure%, :idneysfailure%, :idneys

. ;tro:es;tro:es

. (leeding tendency(leeding tendency


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#olycythemia 0era#olycythemia 0era

. Diagnostic +estsDiagnostic +ests

. (lood cell counts and(lood cell counts andhematocrit mar:edlyhematocrit mar:edlyeleatedeleated

. Hyperuricemia due toHyperuricemia due tohigh cell destructionhigh cell destruction

. (one marro'(one marro'hypercellularhypercellular

Hgb leels to G g/dlHgb leels to G g/dlHct of E or Hct of E or

R(# count of 4 mil/mm7R(# count of 4 mil/mm7


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7ol'c'themia (era "7)7ol'c'themia (era "7)CollaboratieCollaboratiemanagement*management*

.

. P$lebotomy (treatment)P$lebotomy (treatment)bloo" "ra/ingbloo" "ra/ing

. ncrease $y"rationncrease $y"ration. Anticoagulants are !artAnticoagulants are !artof t$era!y to !reentof t$era!y to !reentclot formationclot formation

. C$emot$era!y toC$emot$era!y to

su!!ress bone marro/su!!ress bone marro/actiityactiity

. Radiation therapyRadiation therapy

. (one marro'(one marro'

transplantationtransplantation. ;ignificant number of;ignificant number of

indiiduals 'ith P$ go onindiiduals 'ith P$ go onto deelop acuteto deelop acute

leu:emialeu:emia

7 l th i "7)7 l th i "7)


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N323 Module B part I //

7ol'c'themia (era "7)7ol'c'themia (era "7)

. #lient education guide#lient education guide

. Drin: at least 7 9 dayDrin: at least 7 9 day

. Aoid tight orAoid tight orconstrictie clothing,constrictie clothing,especially garters orespecially garters orgirdlesgirdles

. Jear gloes 'henJear gloes 'henoutdoors in temperatureoutdoors in temperaturelo'er than 1 degreeslo'er than 1 degrees

. #ontact physician first#ontact physician firstsign of infectionsign of infection

. Lse soft?bristledLse soft?bristledtoothbrushtoothbrush

. Do not floss teethDo not floss teeth

. +a:e anticoagulants as+a:e anticoagulants asprescribedprescribed

. Jear support hoseJear support hose'hile a'a:e and up'hile a'a:e and up

. 5leate feet 'hen you5leate feet 'hen you

are seatedare seated. 5xercise slo'ly and only5xercise slo'ly and only

on the adice of youron the adice of yourphysicianphysician

.;top actiity at the;top actiity at thefirst sign of chest painfirst sign of chest pain

. Lse electric shaerLse electric shaer

hite Blood Cell isorderhite Blood Cell isorder


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N323 Module B part IN323 Module B part I /0/0

hite Blood Cell isorder hite Blood Cell isorder

3eukemiaA group of malignant disorders affecting the'hite blood cells and infiltrates tissues of8??(one marro'??9ymph system??;pleen??(rain, M other organs

9eu:emia cells ultimately occupy bone marro' andcirculate through the blood stream&


68/120

N323 Module B part I /=

Deu$emia

;ccurs in all ae roups

Accumulation of d'sfunctional cells due to lossof reulation in cell di(ision

:atal if untreated ;ften thouht of as a childhood disease

he num!er of adults affected is 1? times thatof children

No sinle causati(e aent < Com!ination of enetic and en(ironmental

influences


69/120

Deu$emias are rouped into t'pes444


70/120


p 'p

accordin to ho+ Guic$l' the' proress

and the t'pe of cell in(ol(ed.

&% Acute 9ymphocytic 9eu:emia "A99%))"malignant cells are mainly( lymphocytes%

?? most common in children

??;igns and symptoms may appearabruptly3 Feer

3 (leeding

3 #; manifestations, common

2&% Acute !yeloid"myelogenous% 9eu:emia "A!9% ! l bl t ff t d t


71/120


!yeloblasts affected 0 precursor togranulocytes

" malignant cellsgranulocytes "neutrophils, eosinophils,

basophils%

Also called acute nonlymphoblastic leu:emia"A99%

??stem cell of J(# proliferates, decreasingstem cells aailability for R(# and platelets

??result hyperplasia of bone marro'

??-E of all leu:emias3 GE of the acute leu:emias in adults

??Abrupt, dramatic onset3 ;erious infections or abnormal bleeding


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. 7&% #hronic 9ymphocytic 9eu:emia "#99% ??most common form of adult leu:emia

" malignant cells are ( lymphocytes%

) 9ymph node enlargement is present throughoutbody3 Increased incidence of infection

Pain, paralysis from pressure caused by enlarged lymph

nodes occurs in later stage of disease


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. 2&% #hronic !yeloid 9eu:emia "#!9%??? "malignant cells are granulocytes0eosinophis, basophils, neutrophils%

)Philadelphia chromosome 3


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nclassified Deu$emias

9u!t'pe cannot !e identified

< Malinant leu$emic cells ma' ha(e

characteristics of

D'mphoid M'eloid

Mi-ed

:reGuentl' these patients do not respond+ell to treatment

< 7oor pronosis


75/120



76/120

N323 Module B part IN323 Module B part I 0/0/

. Clinical /anifestationsClinical /anifestations 8aboratory Assessment8aboratory Assessment

o dianose and classif'o dianose and classif'

< 7eripheral !lood e(aluation7eripheral !lood e(aluation

< Bone marro+ e(aluationBone marro+ e(aluation

Definiti*e test: e+aminationDefiniti*e test: e+amination

of cells obtained fromof cells obtained from

bone marro& aspirationbone marro& aspiration

and biopsyand biopsy

Deu$emiaDeu$emia


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Deu$emiaDeu$emia

Collaborati%e CareCollaborati%e Care.


78/120


C$=MC$=M

? @ ? @ phases:phases:11stst phasephase *induction*inductiontherapyther apy,,

00007atient ma'7atient ma'

!ecome criticall' ill!ecome criticall' ill 7ro(ide7ro(ide

ps'choloicalps'choloical

support as +ellsupport as +ell 00common &'=*00common &'=*

22nn phase Intensi!icationphase Intensi!ication

""""Intensification therap'Intensification therap'

< 6ih4dose therap'6ih4dose therap'

< Ma' !e i(en afterMa' !e i(en afterinduction therap'induction therap'

< 9ame drus at9ame drus athiher doseshiher dosesandor other drusandor other drus


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r"r" !$ase!$aset$era!y.Consoli"ationt$era!y.Consoli"ation

therapytherapy, another course of, another course ofchemotherapy single orchemotherapy single orrepeated for ?- yearsrepeated for ?- years

??intent is to cure??intent is to cure

??occurs after remission is??occurs after remission isachieedachieed

. 5liminate remaining5liminate remainingleu:emic cells thatleu:emic cells thatmay not bemay not bepathologically eidentpathologically eident

@th@th phase therapy#phase therapy#

$aintenance$aintenance purpose purpose

is to maintain remission.is to maintain remission.

0000Do+er doses of theDo+er doses of the

same drusame dru

00008oal is to $eep the8oal is to $eep the!od' free of leu$emic!od' free of leu$emic

cellscells

0000Not all leu$emiaNot all leu$emia

respond torespond tomaintenance therap'maintenance therap'


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N323 Module B part I =?

Collaboratie treatmentsCollaboratie treatmentsfor Leuemiafor Leuemia

Drug therapy for infectionDrug therapy for infectionmay include antibacterial,may include antibacterial,antiiral, or antifungal&antiiral, or antifungal&

))))serious aderse effects serious aderse effects

includeincludeototoxicity "disruptionototoxicity "disruptionof hearing and orof hearing and orbalance%, nephrotoxicitybalance%, nephrotoxicity

"disruption of :idney"disruption of :idneyfunction% function%

9nalgesics9nalgesics

Radiation Radiation to areas ofto areas oflymphocytic infiltrateslymphocytic infiltrates

Transfusions of hole bloodTransfusions of hole bloodor blood fractionsor blood fractions

Monitor vital signs and assessMonitor vital signs and assessfor fever. 9 temperaturefor fever. 9 temperatureelevation of A./ degrees Belevation of A./ degrees B

above baseline is significantabove baseline is significantfor a client ith leukopeniafor a client ith leukopeniaand indicates an infectionand indicates an infectionuntil proven otherise.until proven otherise.

%olla&orati'e treatments !or Leemia%olla&orati'e treatments !or Leemia


81/120

N323 Module B part I =1

Bone Marro+ ransplantation "9temBone Marro+ ransplantation "9tem

cell)cell) ne healthy bone marro is given to thene healthy bone marro is given to the

client, hich begins the process ofclient, hich begins the process of

hemotopoiesishemotopoiesis, hich results in normal,, hich results in normal, properly functioning cells, and ideally, a properly functioning cells, and ideally, a

permanent cure. permanent cure.

a.- 9llogenica.- 9llogenic b.- 9utologous b.- 9utologous


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c.- &yngeneicc.- &yngeneic ?bone marro from human ?bone marro from human

leukocyte antigen $39-0matched identicalleukocyte antigen $39-0matched identical

tintin

&uccess depends on the accuracy of the tissue&uccess depends on the accuracy of the tissue

match using human leukocyte antigen $39-match using human leukocyte antigen $39-

Chemotherapy and radiation are used toChemotherapy and radiation are used to

prepare the recipients bone marro for prepare the recipients bone marro fortransplantationtransplantation

Bone Marro+ ransplantationBone Marro+ ra

nsplantation


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Bone Marro+ ransplantationBone Marro+ ransplantation

Procedure8Procedure8

Donor stem cells infusedDonor stem cells infusedintraenously into theintraenously into theblood of the recipientNblood of the recipientN

AntireBectionAntireBectionmedications for onemedications for one

year and then year and thendiscontinued&discontinued&

#ommon complications8#ommon complications8

#onditioning regiment#onditioning regiment

before transplantationbefore transplantation Purpose?Purpose?

&%&%

-&%-&%

5ngraftment5ngraftment**successful ta:e ofsuccessful ta:e oftransplanted cells intransplanted cells inclient@s bone marro'&client@s bone marro'&

G?- days if occursG?- days if occursJ(#, R(#, and plateletJ(#, R(#, and plateletcount begin to risecount begin to rise

1one /arro& Transplantation:1one /arro& Transplantation:


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N323 Module B part IN323 Module B part I ==

1one /arro& Transplantation:1one /arro& Transplantation:

o*er*ie&o*er*ie&

Standard treatment for leu"emia

#urges present marro& of the leu"emic cells

After conditioning, ne&, healthy marro&

gi*en to the client to&ard a cure Sources of stem cells

Conditioning regimen

Transplantation

Ri " f % ;Ri " f % ;


85/120


Ris" for %n;uryRis" for %n;ury

Nadir:Nadir:

period ofperiod of

greatest bone marro&greatest bone marro&suppressionsuppression

1leeding precautions1leeding precautions

!atigue!atigue %nter*entions:%nter*entions:

< Diet therapyDiet therapy

< 1lood replacement1lood replacement

therapytherapy < Drug therapyDrug therapy

< nergy conser*ationnergy conser*ation

%nfection is a ma;or cause of death%nfection is a ma;or cause of deathsepsis is a commonsepsis is a common

complication>/inimal bacteria diet? &ithout/inimal bacteria diet? &ithoutuncoo"ed foodsuncoo"ed foods

/onitoring of daily laboratory/onitoring of daily laboratoryresultsresults Assessment of *ital signsAssessment of *ital signs S"in care, respiratory careS"in care, respiratory care

Nursin Measures for clients +ith immunocompromised

client


86/120

N323 Module B part I =/

client% Place in private room henever possible

% Clean room daily

% Dood hand ashing before and after care

% 3imit using EcommonF e8uipment if at all possible. +eep fre8uently use

e8uipment in the room. Gtili5e disposable eating utensils.

% 3imit number of health care orkers entering the clients room.

% Inspect I( sites, ounds, for signs of infection.


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client underoin !one marro+, stem cell

transplant

Infection protection "p 01 )

Bleedin precautions "p. 0?0)

Ener' manaement "p. )

6ome care of central (enous catheter

"p. )

!alignant 9ymphomas!alignant 9ymphomas


88/120

N323 Module B part IN323 Module B part I ====

!alignant 9ymphomas!alignant 9ymphomas

D'mphomas are cancers "malinancies) of one t'pe ofD'mphomas are cancers "malinancies) of one t'pe of

BCBC called l'mphoc'tes.called l'mphoc'tes. D'mphomas are solidD'mphomas are solidtumors in(ol(in l'mph nodes intumors in(ol(in l'mph nodes in l'mphatic s'stem and in!lood4formin orans

he 2 main t'pes of l'mphoma are 6od$inOs l'mphomahe 2 main t'pes of l'mphoma are 6od$inOs l'mphoma"6D) and non46od$inOs l'mphoma "N6D)."6D) and non46od$inOs l'mphoma "N6D).

Non46od$inOs l'mphoma is more common thanNon46od$inOs l'mphoma is more common than

6od$inOs l'mphoma6od$inOs l'mphoma % diagnosis and staging of diseasediagnosis and staging of disease is based on ais based on a

biopsies, CTs abd'chest, 3BTs, bone marro biopsies biopsies, CTs abd'chest, 3BTs, bone marro biopsies

D'mphomas


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D'mphomas

D'mphatic s'stem4 net+or$ of l'mphatic (esselsare l'mph nodes, +hich house collections ofl'mphoc'tes.

D'mphoc'tes that !ecome cancerous"l'mphoma cells) ma' remain confined to a

sinle l'mph node or ma' spread to the !onemarro+, the spleen, or (irtuall' an' other oran

D'mphomas can de(elop from either B or l'mphoc'tes.

T lymphocytes are important in reulatin theimmune s'stem and in fihtin (iral infections.

B lymphocytes produce anti!odies.

the l'mphatic s'stem


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' p '

:CN transports fluids throuhout the !od'.

consists of thin4+alled l'mphatic (essels,l'mph nodes, and t+o collectin ducts

he fluids contain proteins, minerals,

nutrients, and other su!stances, +hichpro(ide nourishment to tissues.

important functions of the l'mphatics'stem are to remo(e damaed cells from

the !od' and to pro(ide protection aainstthe spread of infection and cancer.

4.-4.- $odgkins 3ymphoma$od

gkins 3ymphoma !eed0&ternberg!eed0&ternberg


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N323 Module B part IN323 Module B part I >1>1

4.-- $odgkins 3ymphomag y p !eed &ternberg gspecific cancer cell type-specific cancer cell type- is one of the mostis one of the mostcurable types of cancer.curable types of cancer.

occurs in people in their mid to late As and inoccurs in people in their mid to late As and in people older than /A people older than /A causes unknon, factors in development maycauses unknon, factors in development may

include viral infection =:(-, and e#posure toinclude viral infection =:(-, and e#posure to

chemicals, to#inschemicals, to#ins.- Non $odgkins 3ymphoma 4 subtypes-.- Non $odgkins 3ymphoma 4 subtypes- si#th most common cause of cancer in the Gnitedsi#th most common cause of cancer in the Gnited

&tates&tates

more common in men, hite individuals, and peoplemore common in men, hite individuals, and peopleolder than /A years of age.older than /A years of age. Causes7Causes7 see above, immunosuppressive medications,see above, immunosuppressive medications,

chemo, radiation, e#posure carcinogenschemo, radiation, e#posure carcinogens

Hodg"ins 8 mphoma


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N323 Module B part I >2

Hodg"ins 8ymphoma Cancer that starts in a single lymph node or a

single chain of nodes, e*entually infiltratesother organs)lungs, spleen, li*er

/ar"er: Reed-Sternberg cell Signs and Symptoms: 8arge, painless lymph

node usually in the nec", a+illary, or inguinal,

fe*er, malaise, night s&eats, &eight loss, fe*er 6ther s9s depends on stage of disease One of the most curable cancers Disease belo& diaphragm can spread to li*er,

abo*e diaphragm confined to lymph nodes fora time<

Treatment: e+ternal radiation alone or &ithcombination chemotherapy

$ H d "i 8 h$on Hodg"ins 8ymphoma


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N323 Module B part IN323 Module B part I >3>3

$on-Hodg"ins 8ymphoma$on-Hodg"ins 8ymphoma

All lymphoid cancers that do not ha*e the Reed-All lymphoid cancers that do not ha*e the Reed-

Sternberg cellSternberg cell

/ore than 23 types of non-Hodg"ins lymphoma/ore than 23 types of non-Hodg"ins lymphoma

1ur"itts, 8arge 1 Cell lymphoma highly aggressi*e1ur"itts, 8arge 1 Cell lymphoma highly aggressi*e

diseasesdiseases 8o&-grade lymphomas less responsi*e to treatment@8o&-grade lymphomas less responsi*e to treatment@

cures are rarecures are rare

Treatment:Treatment: radiation therapy and multi agentradiation therapy and multi agent

chemotherapy, or single-agent therapychemotherapy, or single-agent therapy

4taging of o"gin Lym!$oma4taging of o"gin Lym!$oma


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4taging of o"gin Lym!$oma4taging of o"gin Lym!$oma

an" 5on+o"ginan" 5on+o"gin

n 1 lym!$ region only

n 6 2 lym!$ regions on t$e same si"e oft$e "ia!$ragm

n t$e lym!$ no"es, s!leen, or bot$ an" onbot$ si"es of t$e "ia!$ragm

V 3-trano"al inolement (eg, bone marro/,lung, lier)

/ultiple /yeloma (plasma cell myeloma


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/ultiple /yeloma (plasma cell myeloma #lasma cell disorder, &hite blood cell cancer

that in*ol*es an o*ergro&th 1 lymphocyte cell damages bone marro& 4 bone

e+cess of antibodies and e+cess cyto"inescauses a progressive bone destruction,

bleeding problems, kidney failure,immunosuppression.

/ultiple tumors de*elop in *ertebrae, ribs,pel*is, 4 s"ull

89T pathologic or spontaneous !B,hypercacemia de*elops D9T bone brea"do&n

ncommon cancer


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N323 Module B part I >/

Cause: un"no&n, radiation and chemical

e+posure may be ris" factors /anifestations: fatigue, easy bruising, bone

pain, fractures, hypertension, increased

infection, hypercalcemia, and fluid imbalance

Diagnosis: bone marro& biopsy (thinning,

>S&iss cheese? 1ence-ones? protein in

urine

Treatment: chemotherapy, bone marro&transplant, pain management, bone fracture

pre*ention, blood transfusion, hydration

#l t l t Di d 4 1l d


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N323 Module B part I >0

#latelet Disorders 4 1lood

Clotting Disorders

Autoimmune


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N323 Module B part I >=

Autoimmune

Thrombocytopenic #urpura %T# >%diopathic thrombocytopenic purpura? prior name Autoimmune disorder, autoantibodies are

produced

Thrombocytes (plateletes are recogniEed as

foreign causing macrophages to destroy them -- trigger un"no&n

1one marro& production of platelet is normal,

platelet destruction is increased, leading tolo& number of circulating platelets

Autoimmune


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N323 Module B part I >>

Autoimmune

Thrombocytopenic #urpura /anifestations: 8arge ecchymosis or

petechial rash on arms, legs, upper chest, and

nec"

Se*ere complications may include renal failure,

/%, stro"e

Diagnosed: by decreased platelet count and

large numbers of mega"aryocytes in the bone

marro&,

antiplatelet antibodies may be present

Autoimmune


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N323 Module B part I 1??

Autoimmune

Thrombocytopenic #urpura (Continued %nter*entions include:

< Therapy to pre*ent bleeding

< Drug therapy to suppress immune function

(corticosteroids, %muran < 1lood replacement therapy (platelet

transfusion especially &ith counts less than

3F, FFF9cubic mm

< /aintain safe en*ironment to reduce ris"bleeding

< Splenectomy

hrom!otic hrom!oc'topenichrom!otic hrom!oc'topenic


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N323 Module B part IN323 Module B part I 1?11?1

7urpura "7)7urpura "7)

Rare disorder@ platelets clump abnormally togetherRare disorder@ platelets clump abnormally togetherin the capillaries, results in bleeding problemsin the capillaries, results in bleeding problems

else&here, pt< may ha*e hemolytic d9o, neurologicelse&here, pt< may ha*e hemolytic d9o, neurologic

abnormalities,abnormalities,

%nappropriate clotting &hen trauma occurs%nappropriate clotting &hen trauma occurs Cause:Cause: Autoimmune reaction in blood *esselsAutoimmune reaction in blood *essels

causes clumping, certain drug to+icitiescauses clumping, certain drug to+icities

Results in tissue ischemia


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7urpura "7)7urpura "7)

Colla!orati(e manaementColla!orati(e manaement 9top autoimmune process to pre(ent clottin and9top autoimmune process to pre(ent clottin and

clumpin, and decreased platelets circulatin "!leedin)clumpin, and decreased platelets circulatin "!leedin)

7lasma pheresis, fresh froHen plasma transfusion7lasma pheresis, fresh froHen plasma transfusion

rus platelet inhi!itors < A9A, 7rostin, 7licam'cinrus platelet inhi!itors < A9A, 7rostin, 7licam'cin"antineoplastic, anti!iotic)"antineoplastic, anti!iotic)

7latelets enerall' contraindicated*ma' lead to ne+7latelets enerall' contraindicated*ma' lead to ne+

(: platelet comple-es and increased clottin(: platelet comple-es and increased clottin

ImmunosuppressantsImmunosuppressants

Heparin-induced thrombocytopeniaHeparin-induced thrombocytopenia "6I)"6I)

" )


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+ith or +ithout+ith or +ithout thrombosisthrombosis "6I)"6I) Q+hite clotQ+hite clots'ndrome&s'ndrome&

isis throm!oc'topeniathrom!oc'topenia "lo+"lo+ plateletplatelet counts)counts) due to thedue to theadministration ofadministration of heparinheparin

mainl' associated +ith unfractionated heparinmainl' associated +ith unfractionated heparin":6), it can also occur +ith e-posure to":6), it can also occur +ith e-posure to

lo+4molecular +eiht heparinlo+4molecular +eiht heparin "DM6), at lo+er"DM6), at lo+erratesrates

espite the lo+ platelet count, it is aespite the lo+ platelet count, it is a throm!oticthrom!otic disorder, +ith (er' hih rates ofdisorder, +ith (er' hih rates of throm!osisthrom!osis, in, in

thethe arteriesarteries +ith or +ithout+ith or +ithout (enous(enous complicationscomplications

most common complication most common complication

http://en.wikipedia.org/wiki/Thrombocytopeniahttp://en.wikipedia.org/wiki/Thrombocytopeniahttp://en.wikipedia.org/wiki/Platelethttp://en.wikipedia.org/wiki/Platelethttp://en.wikipedia.org/wiki/Heparinhttp://en.wikipedia.org/wiki/Heparinhttp://en.wikipedia.org/wiki/Low-molecular_weight_heparinhttp://en.wikipedia.org/wiki/Low-molecular_weight_heparinhttp://en.wikipedia.org/wiki/Thrombosishttp://en.wikipedia.org/wiki/Thrombosishttp://en.wikipedia.org/wiki/Thrombosishttp://en.wikipedia.org/wiki/Thrombosishttp://en.wikipedia.org/wiki/Arteryhttp://en.wikipedia.org/wiki/Arteryhttp://en.wikipedia.org/wiki/Veinhttp://en.wikipedia.org/wiki/Veinhttp://en.wikipedia.org/wiki/Veinhttp://en.wikipedia.org/wiki/Arteryhttp://en.wikipedia.org/wiki/Thrombosishttp://en.wikipedia.org/wiki/Thrombosishttp://en.wikipedia.org/wiki/Low-molecular_weight_heparinhttp://en.wikipedia.org/wiki/Heparinhttp://en.wikipedia.org/wiki/Platelethttp://en.wikipedia.org/wiki/Thrombocytopenia


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'picall' occur after a!out fi(e da's oftherap'

can appear sooner in patients

pre(iousl' treated +ith heparin. Associated disorders , IC,

pulmonar' em!olism, cere!ral

throm!osis, m'ocardial infarction, andischemic in5ur' to the les or arms,

I


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N323 Module B part IN323 Module B part I 1?1?

'pe I'pe I

7atients ha(e a transient decrease in platelet7atients ha(e a transient decrease in platelet

count +ithout an' further s'mptoms.count +ithout an' further s'mptoms. 'pe II'pe II

CauseCause autoimmuneautoimmune alleric reaction to heparinalleric reaction to heparin

After heparin is administered to a patient, an After heparin is administered to a patient, animmune comple-immune comple- can form !et+een heparin andcan form !et+een heparin anda specific !lood factor "plateleta specific !lood factor "platelet factor , or 7:)factor , or 7:)that is released !' platelets.that is released !' platelets.

http://en.wikipedia.org/wiki/Autoimmune_disorderhttp://en.wikipedia.org/wiki/Autoimmune_disorderhttp://en.wikipedia.org/wiki/Autoimmune_disorder


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he !od'he !od'

(ie+s this heparin47:(ie+s this heparin47:comple- as a forein su!stance.comple- as a forein su!stance.herefore,herefore, an anti!od' is formed aainstan anti!od' is formed aainst

the heparin47: comple-.the heparin47: comple-.

he anti!od'he anti!od' !inds to this comple- and!inds to this comple- andthe platelets are destro'edthe platelets are destro'ed

N323 Module B part IN323 Module B part I 1?/1?/

Heparin-induced thrombocytopeniaHeparin-induced thrombocytopenia "6I)"6I)


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+ith or +ithout+ith or +ithout thrombosisthrombosis "6I)"6I)


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his disruption of platelets can lead to thehis disruption of platelets can lead to the

formation of ne+formation of ne+ !lood clots in patients +ith!lood clots in patients +ithimmune4mediated 6I.immune4mediated 6I.

he resulthe result can !e a deep (ein throm!osis "incan !e a deep (ein throm!osis "in

the (eins of the thih or the (eins of the thih or pel(is), pulmonar'pel(is), pulmonar'em!olism, or e(en a heart attac$ or stro$e.em!olism, or e(en a heart attac$ or stro$e.

44Clot formation is mainl' arterial and rich in44Clot formation is mainl' arterial and rich inplatelets "+hite clot s'ndrome)platelets "+hite clot s'ndrome)

44D throm!oc'topenia and platelet4fi!rin44D throm!oc'topenia and platelet4fi!rinthrom!ithrom!i

N323 Module B part IN323 Module B part I 1?=1?=


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s especiall' in lo+er e-tremities ma's especiall' in lo+er e-tremities ma'occur occur

s$in lesions and necrosis ma' also occurs$in lesions and necrosis ma' also occur

at the site of the heparin infusionat the site of the heparin infusion 6I occurs more commonl' in surical6I occurs more commonl' in surical

settins rather than non4surical settinssettins rather than non4surical settins

N323 Module B part IN323 Module B part I 1?>1?>

ianosisianosis


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N323 Module B part IN323 Module B part I 11?11?

44decreased platelet counts44decreased platelet counts

44anti!od' for 6I44anti!od' for 6I reatmentreatment

44C 6eparin, use other drus44C 6eparin, use other drus

44Refludan "lepirudin), arotro!an44Refludan "lepirudin), arotro!an "throm!in"throm!ininhi!itors)inhi!itors)

4444:ondaparinu- "Ari-tra),:ondaparinu- "Ari-tra), ":actor inhi!itor)":actor inhi!itor)

9e(ere clottin pro!lems*plasmapheresis9e(ere clottin pro!lems*plasmapheresis

7rotamine sulfate < antidote to 6eparin7rotamine sulfate < antidote to 6eparin

9plenectom'9plenectom'

Hemophilia and Von Willebrand diseaseHemophilia and Von Willebrand disease


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*emophilia*emophilia ? ? It is an )0linked recessiveIt is an )0linked recessive

bleeding disorder. $emophilia is a bleeding bleeding disorder. $emophilia is a bleedingdisorder caused by a deficiency in one of todisorder caused by a deficiency in one of to

blood clotting factors7 factor (III or factor I). blood clotting factors7 factor (III or factor I).

There are to forms of hemophilia.There are to forms of hemophilia.$emophilia 9$emo philia 9 classic hemophilia-, hichclassic hemophilia-, hich

accounts for about 2AH of all cases, is aaccounts for about 2AH of all cases, is a

deficiency in clotting factor (III.deficiency in clotting factor (III.


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$emophilia :$emo philia : Christmas disease- is aChristmas disease- is adeficiency in clotting factor I).deficiency in clotting factor I).

They are recessive se#0linked, hich meansThey are recessive se#0linked, hich means

that the gene abnormalities are inheritedthat the gene abnormalities are inheritedthrough the mother and that nearly everyonethrough the mother and that nearly everyone

ith hemophilia is male.ith hemophilia is male.

on ille!rand iseaseon ille!rand isease


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on ille!rand iseaseon ille!rand isease

. JF,JF, a enetic !lood disorder that affectsa enetic !lood disorder that affectsclottinclottin

his l'coprotein is deficient or defecti(ehis l'coprotein is deficient or defecti(e

inin *on illebrand*on illebrand diseasedisease DD ariable factor $III deficienciesariable factor $III deficiencies

and platelet dysfunctionand platelet dysfunction

. Autosomal dominant, seen in both sexesAutosomal dominant, seen in both sexes

"iagnosis7"iagnosis7 development of a large cephalohematomadevelopment of a large cephalohematoma


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or intracranial hemorrhage after birth, a history ofor intracranial hemorrhage after birth, a history of

prolonged bleeding after trauma could be indicative prolonged bleeding after trauma could be indicative

of clotting disorder that needs further evaluation.of clotting disorder that needs further evaluation.

Treatment7Treatment7 hemophilia is not curable, but treatmentshemophilia is not curable, but treatments

that includes cryoprecipitate, or replacement of factorthat includes cryoprecipitate, or replacement of factor

(III, or I) , ""9(P(III, or I) , ""9(P synthetic vasopressin stimulates increase insynthetic vasopressin stimulates increase infactor (III, (6B-factor (III, (6B- to increase clotting factor levels canto increase clotting factor levels can

prevent crippling deformities and prolong life prevent crippling deformities and prolong life

e#pectancy.e#pectancy.

009ntifibrinolytic therapy?Cyclokapron, 9micar 009ntifibrinolytic therapy?Cyclokapron, 9micar

isseminated Intra(ascular Coaulation"IC)


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. Inoles both 8 ;erious bleeding and

thrombotic "clotting% D/6. DI# is a life threatening disorder

that occurs as a complication of

diseases and conditions thataccelerate clotting, 9/+ depletion ofplatelets M clotting factors and

eentually 9/+ uncontrollablehemorrhage occurs&

isseminated Intra(ascular Coaulation"IC)


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N323 Module B part I 11/

A& #auses8 infection, septecemia, iral, fungal,

ric:ettsial, protooal infections, obstetricalcomplications, neoplastic diseases, burns,trauma, heatstro:e, or any other number ofdisorders&

. (& Pathophysiology8 unclear 'hy certaindisorders lead to DI#& +he triggering


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d sorders lead to DI#& he tr gger ngmechanism is by the entrance of a foreign

protein "infection% , particularly gram?negatieinfection

. 9/+ endotoxins into the circulation and causeascular endothelial essel inBury

"microascularcirculation% 39/+ trigger clottingmechanism throughout body?9/+ comsumptionof clotting factors and fibrinolysis occurs

. 9/+ hemorrhage, and eentually hypotension M

shoc:


Accelerated clotting, actiation of


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Accelerated clotting , actiation ofprothrombin, excess thrombin

+hrombin conerts fibrinogen to fibrin

Fibrin clots in microcirculation Hypofibrinogenia, hypoprothrombinemia,

thrombocytopenia, deficiencies in factors

$ and $III Fibrinolytic system actiates dissoles

fibrin clots H5!6RRHA


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. Diagnostic +ests. P++/P+ prolonged

. Reduced fibrinogen, antithrombin, andplatelets

. 5leated fibrin?split products, and eleatedD?dimers "cross lin:ed fibrin fragments%

. Decreased leels of factors $, $II, $II, C,CIII

isseminated Intra(ascular

Coaulation"IC)


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Coaulation"IC)

. #linical Findings8

abnormal bleeding any'here in the body,cutaneous ooing of serum, cyanoticextremities, petechia, restlessness, anxiety

. #omplications8 acute tubular necrosis, shoc:,multi organ failure

. Interentions/+reatment8 treatment ofunderlying disorder, administration of blood,fresh froen plasma, platelet, pac:ed R(#s,heparin therapy "controersial%

002 kuliah nutrisi untuk hematopoiesis (3)

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