kp 1 pengantar blok 2.4 hematolimfopoietik.ppt

7/29/2019 KP 1 Pengantar Blok 2.4 Hematolimfopoietik.ppt

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HEMATOLIMFOPOIETIKBLOK 11

FAKULTAS KEDOKTERAN

UNIVERSITAS ANDALAS

PADANG

2008/2009


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Tim Pengelola Blok 11

KORDINATOR 1 : Dr Irza Wahid SpPD KHOM

WAKIL KORDINATOR : Prof DR Dr H Ellyza Nasrul SpPK (K)

Staf bidang materi : - Prof Dr H Nusirwan Acang DTM&H SpPD KHOM- Dr H Firman Arbi SpA (K)

Staf bidang Skill / Psikomotor : - Prof Dr Hanifah Maani SpPK (K)

- Dr Tuti Prihandani SpPK

Staf Bidang Monitoring / Evaluasi / Administrasi

- Dr Efrida SpPK M Kes

- Dr Zelly Dia Rofinda SpPK


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Nama : Dr Irza Wahid SpPD KHOM

Tempat / Tanggal Lahir : Padang / 23 November 1967

Alamat : Jalan Kolam Indah Raya No A6 Cendana Mata Air Padang

Telp. : 07516195208126605439

Pekerjaan : Staf Subagian HematologiOnkologi MedikBagian Ilmu Penyakit dalam FK Unand / RS Dr M Djamil

RIWAYAT PENDIDIKAN

No

Nama Pendidikan Nama Sekolah Waktu

1 SD SD Yos Sudarso Padang 1973 -1980

2 SMP SMP Negeri 1 Padang 1980 -1983

3 SMA SMA Negeri 1 Padang 1983 -1986

4 Kedokteran Umum FK Unand Padang 1986 1993

5 Program Pendidikan Dokter

Spesialis I Ilmu Penyakit Dalam

FK Unand / BLU RS Dr M Djamil

Padang

01/07/1998 10/07/ 2003

6 Program Awal Pendidikan Dokter

Spesialis II Konsultan Hematologi

Onkologi Medik

FK Unand / BLU RS Dr M Djamil

Padang

FKUI / RSCM / RSKD Jakarta

01/01/2004 31/12/2006

7 Program S3 Biomedik FK Unand FK Unand 01/07/2008 sekarang


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Modul 1 : anemia defisiensi dan aplastik.

Modul 2 : anemia hemolitik dan pasca perdarahan.

Modul 3 : gangguan hemostasis.

Modul 4 : leukemia.

Modul 5 : limfadenopati.

Modul 6 : transfusi darah.

Skills labs : Uji Rumple Leed

: Flebotomi

Praktikum : Hematologi

: Patologi Anatomi


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TOPIK PRAKTIKUM BLOK XI

Minggu I. Anemia

Evaluasi darah tepi dan sumsum tulang

1.Anemia defisiensi Fe

2.Anemia aplasi

3.Anemia hemolitik

Minggu II. Hitung eritrosit dan retikulosit

Minggu III. Bleeding Time (cara Ivy), Retraksi bekuan dan

Golongan Darah

Minggu IV. Evaluasi darah tepi dan SST : ALL (L1-L3) dan CLLMinggu V. Evaluasi darah tepi dan SST : AML (M1-M7) dan

LGK

Minggu VI. Limfadenopati (PA)


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Hematopoeitik :Sum-sum tulang --------------------------------> Darah tepi

Pluripotent stem cell

I. Myeloid progenitor cellA.Erythro-MKprogenitor cellEritropoesiseritrosit Anemia / PolisitemiaMegakariopoesistrombosit Trombositosis / Trombositopenia

B.Gran-monoprogeni tor cellGranulopoesis leukosit Leukopenia / LeukositosisMonositopoesis monosit Monositopenia / Monositosis

II. Lymphoid progenitor cellLimfopoesislimfosit Limfositopenia / Limfositosis

sel plasma

Extramedular ------------------------------------> Hati / Limpa


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ANEMIA Parameter : Kadar hemoglobin Metode Sahli

Pria dewasa : Wanita dewasa : Hamil : Hb < 13 : < 12 : < 11 gr %

Gejala dan tanda

Hb hipoksia kompensasi kardiovaskular

* Pucat * angina pektoris * kardiomegali

Mukosa * claudicatio intermiten * palpitasi

Kulit * tinitus * dispneu

* berkunang * bising sistolik

* cepat lelah * gagal jantung Gradasianemia ringan : sedang : berat : > 8 : 6 8 : < 6 gr %

Morfologi mikro / normo / makrositer -- hipo/normo/hiperkrom

Patofisiologi defisiensi aplastik hemolitik perdarahan

Etiologi Cacing, low intake, kelainan imun, trauma


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LEUKEMIADefinisi : Proliferasi abnormal dari dari sel induk darah

Serial Mieloid Serial LimpoidLeukemia mieloblastik akut Leukemia limfoblastik akut( Tu. Dewasa muda ) ( Tu. Anak-anak )

Leukemia mielositik kronik Leukemia limfositik kronik

( dewasa muda + orang tua ) ( Semua umur )

Etiologi : Unknown Virus, radiasiGejala klinis : - Anemia

- Infeksi

- Trombositopenia

- Hiperkatabolikhiperurisemia- Infiltrasi organorganomegali, hipertrofi gusi,

meningeal, cerebral

Diagnosis : Hapusan darah tepi blast > 5 %BMP blast > 30 % / > 20 % (WHO, 2001)Sitokimia, immunophenotype, sitogenetik


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HEMOSTASIS - DIATESIS HEMORAGIS

- TROMBOSIS

Vaskular

Trombosit Koagulasi


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Non HodgkinHodgkin

Proliferation

andTransformation Proliferation

Specific

Non neoplasm / Infection

Non specific

LimfomaSecondary /metatstatic

MalignantBenign

Neoplasm

Lymphadenopathy

LIMFADENOPATI


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TRANSFUSI DARAH

* ERITROSIT PRC, WE, DFRC

*LEUKOSIT

* TROMBOSIT

* PLASMAKRIOPRESIPITAT KONSENTRAT Fc VIII

KRIOSUPERNATAN ALBUMIN, IMUNOGLOBULIN


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REAKSI TRANSFUSI

Definisi :Komplikasi / efek samping yang terjadi akibat

pemberian transfusi

Klasifikasi

Imunologi : Produksi anti bodi terhadap

aloantigen pada eritrosit, leukosit,

trombosit atau protein plasma darah Non Imunologik : Berhubungan dengan bahan

fisika/kimia komponen darah atau

kontaminan


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DAFTAR PUSTAKA

- Greer, Foerster, Lukeni, et al.Wintrobes Clinical Hematology 2thedition, 2004.

- Devita VT, Hellman S, Rosenberg SA, et al. Cancer Principles &

Practice of Oncology 7th edition , 2005

- Colman RW, Marder VJ, Clowes AW, et al.Hemostasis &

Thrombosis Basic Principles & Clinical Practice, 2006

- Sudoyo AW, Setiyohadi B, Idrus A et al. Buku Ajar Ilmu Penyakit

Dalam Jilid 2 Edisi 4 , 2006.

- Waldo E, Nelson RE, Robert K et al. Nelson Text Book of

pediatrics, 17 th edition, 2004.-Permono B, Sutaryo,Ugrasena IDG et al. Buku Ajar Hematologi

Onkologi Anak, 2005.

- Ganda Subrata, Penuntun Laboratorium Klinik


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Factors necessary for

erythropoiesis

1. Erythropoietin

2. Iron

3. Vitamin B12 (cyanocobalamin) 4. Folic Acid (folate)

5. Ascorbic acid (Vitamin C)

6. Pyridoxine (Vitamin B6) 7. Amino acids


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Deficient RBC Production

Neoplasia: covered by another lecturer

Myelofibrosis: Remove spleen; transfusion whennecessary with packed cells.

Pernicious anemia: B12 injections Microcytic (iron deficiency) anemia: iron: only

ferrous iron (sulfate, fumarate, gluconate). Ferric ironis not absorbedliquid Geritol wont do.

Aplastic anemia: stop medication; may have to dobone marrow transplant

Renal disease: Correct if possible, otherwise renaltransplant


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Normoblasts

Pronormoblast


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Regulation of Erythropoiesis

Normal body requirements for oxygen is 250

ml/min

Almost all of that is for use in oxidative

phosphorylation by the mitochondria.

The heart normally pumps out 1,000 ml

oxygen per minute.

Less than 250 ml/min causes tissue hypoxia


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Kidneys receive 25% of cardiac outputapproximately 1,250 ml of blood/minyetit is only 0.5% of body weight.

The 1,250 ml/min contains 250 ml/min ofoxygen

The amount of oxygen/min received by thekidneys is the same as that required by body

tissues/min. Therefore, renal monitoring of oxygen is

logical choice.


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If renal tissue is hypoxic, erythropoietin isproduced by renal peritubular interstitial cells.

The gene for erythropoietin is located on

chromosome 7. Erythropoietin is not the only factor required

for red cell development. We will cover some

of these later.


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While the kidney produces most of theerythropoietin, the liver and other tissues alsoproduce some.

In fetal life, the liver produced all of theerythropoietinas the final kidneythe

metanephros - had not yet completeddevelopment.


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Erythropoietin increases RBC production in 3

ways:

Promotes pronormoblast production

Shortens the transition time through the

normoblast stage

Promotes the early release of reticulocytes.


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How many are produced: 25 billion /24 hours.

The entering cells are reticulocytes which

should be 1% of the total population ofcirculating erythrocytes.

Erythrocytes last 120 days and are destroyed by

the spleen.

Red cell production should equal red cell

destruction.


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Determination of RBC size

MCV = mean corpuscular volume in femtoliters(10-15 liters) abbreviated fl.

This is the most accurate method of measuring redblood cells and is the one done by automatedCoulter counters.

MCH = mean corpuscular hemoglobin does notadd anything to the diagnosis except additionalexpense. It will not be considered further.

eren a agnos s us ng


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eren a agnos s us ngMCV

Macrocytic anemias (MCV = 150 fl)larger than normal cells

Normocytic (MCV = 80-96 fl)cells arenormal in volume.

Microcytic anemias (MCV = 50 fl)cells

are smaller than normal.

ur scuss on o anem a s


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ur scuss on o anem a slimited to:

We will only discuss those anemias that are due toa deficiency of:

Iron

Folic acid Vitamin B12

Vitamin

Or those anemias that will be benefitted by theadministration of erythropoietin.

.


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Iron Iron deficiency is the most common cause ofanemia.

Results in microcytic hypochromic anemia.


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Iron is used in: Hemoglobin. Heme enzymes, e.g., cytochromes, catalase,

peroxidase

Myoglobin Metalloflavoprotein enzymes such as xanthine

oxidase

The mitochondrial enzyme alpha-glycerophosphate

oxidase and other mitochondrial enzymes.

Other enzymes and processes


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Iron in hemoglobin


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Iron in hemoglobin

Iron Deficiency Common


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Iron Deficiency

Common

Causes

Inadequate dietary intake of iron especiallyin:

Infants in the postnatal period

Young females after menarche Adolescents of both sexes

During pregnancy

Iron Deficiency Common


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Iron Deficiency

Common

Causes (cont)

Partial gastrectomy due to ulcers Blood loss due to:

Bleeding peptic ulcers

Malignancy Trauma in which there is excessive bleeding

(includes blood loss through extensive bruising)

Malabsorption syndromes

Cystic fibrosis

Celiac Disease (nontropical sprue)


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Iron Deficiencylead toxicity

Can lead to lead toxicity from pica Iron deficiency enhances uptake of heavy

metals, including lead.

Lead is still used in all outside house paint.

Lead is highly concentrated in the first fewinches of soil in citiesfrom car exhaust of 20years or more ago.

Infants love to each picatheir taste of the world.


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Heme iron and nonheme iron

Heme iron very well absorbed. Present inmuscle as part of myoglobin.

But: Do not eat meat that is bloody.Bacteria love blood and it can be the source

of severe food poisoning.

Nonheme iron not absorbed well but forms

the bulk of dietary iron.


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IronAbsorption

Absorbed from the duodenum (where most

drugs are absorbed) and upper jejunum.

Vitamin C taken with the iron increases

absorption by reducing dietary ferric to ferrous

iron.

Caffeine and other xanthines decrease

absorption.


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IronPreparations available

Only absorbed well in the ferrous(us is forus) form. Ferrous sulphate, ferrous gluconateand ferrous fumarate are three that are

available. Ferric form only 10% absorbedick! Ferric

ammonium citrate is in Geritol Tonic.

However, patients feel goodcould it bebecause of the 12% alcohol content?


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IronNot from Geritol Tonic


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Iron TherapySide effects of

oral preparations Nausea

Gastric discomfort

Constipation

Diarrhea

Good idea to start patients on small doses and

increase gradually. Less side effects that way.


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IronParenteral solution Should be used cautiously because 0.2% to

3% of patients develop hypersensitivityincluding anaphylactic shock to parenteraliron.

Parenteral iron does not appear to provide afaster response than oral preparations.

Only one in Saskatchewan: iron dextran

Reasons for use:

Renal failure

Short bowel

Celiac disease (sprue)

ANEMIA DEFISIENSI


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ANEMIA DEFISIENSIBESI

Metabolisme Besi

Intake( Fe 3+ = Ferri )( daging organ, kacang polong, air tebu, daun sup, kerang )

Fe 3+ Fe 2+ = Ferro

Mukosa duodenum / jejunum prox ( Absorbs i)

Fe 2 + Fe 3+

Transferin ( Transport)

Mioglobin / enzim Hemoglobin(Jaringan) (Eritrosit)

Feritin / hemosiderin ( Storage)

( Sistim RE )


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EtiologiAsupan Kehi langan Fe Kebutuhan meningkat

- malnutrisi - perdarahan - hamil / laktasi- ggn absorbsi - GGK / HD - anak-anak

Patofisiologi

- Def Fe prelaten / deplesi Fe

cad. Fe ,Fe serum dbn- Def Fe laten cad. Fe habis, Hb batas bwh N- Anemia Def Fe

Gejala Klinis- gejala anemia

- gejala defisiensi atrofi papil lidah, stomatitis angularis,disfagia, koilonychia

- gejala penyakit dasar


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DIAGNOSIS* Anemia, retikulosit menurun

* Mikrositer, hipokrom, anulosit

* SI menurun / TIBC meningkat* Transferin < 16 mg %

* Feritin serum < 12 mg %

* SST : Hiperplasia eritropoetik, pengecatan besi ( - )

* Pemeriksaan untuk mencari penyebab

PENATALAKSANAAN* Preparat besi 3 X 200 mgHb normal maintenance 3 X 100 mg (3 bln)

* Pengobatan penyakit dasar

kp 1 pengantar blok 2.4 hematolimfopoietik.ppt

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