pendekatan klinis pansitopenia
TRANSCRIPT
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BAB I
Pendahuluan
Pansitopenia adalah suatu keadaan dimana adanya penurunan jumlah komponen darah
yang terdiri dari 3 macam, yaitu yang terdiri dari Eritrosit (sel darah merah), Leukosit (sel darah
putih), dan trombosit. Ketiga tersebut jika mengalami penurunan akan mengakibatkan terjadinya
anemia, leukopenia dan trombositopenia.
Etiologi terjadinya pansitopenia dapat berupa primer, sekunder, dan infeksi virus. Primer
merupakan yang belum diketahui penyebabnya, bisa berupa idiopatik atau keturunan. Sekunder
yang berasal dari obat-obatan seperti NSAID, Sitotoksik, atau berdasarkan dari obat seperti
respon idiosinkrasi. Sedangkan berdasarkan dari infeksi virus yang sering menyebabkan
terjadinya pansitopenia adalah virus hepatitis B dan C.
Terjadinya penurunan jumlah komponen darah tersebut dapat diketahui melalui
pendekatan dari anamnesis, pemeriksaan fisik dan pemeriksaan penunjang. Pada anamnesis
biasanya didapatkan adanya kelainan seperti anemia aplastic, Sindrom gagal sumsum tulang
turunan, adanya riwayat keguguran, riwayat menderita keganasan, adanya gangguan metabolic,
riwayat penyakit hati dan adanya riwayat penggunaan obat sitotoksik dan radioterapi.
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BAB II
PENDEKATAN DIAGNOSIS PANSITOPENIA
Pansitopenia adalah Penurunan jumlah 3 komponen darah Eritrosit, Leukosit dan Trombosit.
Yang akan mengakibatkan Anemia, leukopenia, dan trombositopenia. Penyebab penurunan
produksi darah di sumsum tulang, Bone marrow faillure syndrome, Immune mediated
destruction,Non immune mediated sequestration in perifer.
Etiologi
Table of etiologies for pancytopenia. Abbreviation: GVHD, graft-versus-host diseaseFrom the collection of Jeff K. Davies
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Derajat pansitopenia
Ringan Hemoglobin 2000/mm , trombosit >20.000/mm
Sedang Hemoglobin
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Pendekatan Diagnosis
Anamnesis
Anemia aplastik
Sindrom gagal sumsum tulang turunan
Riwayat keguguran
Riwayat menderita keganasan
Gangguan metabolik
Riwayat penyakit hati
Riwayat penggunaan obat sitotoksik dan radio terapi
Pemeriksaan fisik
Mata :
Perdarahan retina (trombositopenia)
Infiltrasi lekemik (leukimia akut)
Sklera ikhterik (Paroxysmal nocturna hemoglobinuria, hepatitis, sirosis)
Epiphora (dyskeratosis congenital)
Oral :
Ptekie/ Perdarahan di rongga mulut (trombositopenia)
Stomatitis/chelitis (neutropenia, def. Vit B12)
Hiperplasia gusi (leukimia )
Kandidiasis oral/ eksudat faring (neutropenia, infeksi herpes virus)
Kardiovaskular:
Takikardi, edema, CHF
Bekas pembedahan jantung (sindrom kongenital)
Respiratori :
Clubbing (ca. paru)
Takipnea
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Abdomen :
Nyeri perut kakan atas ( hepatitis )
Limfadenopati (infeksi, penyakit limfoproliferatif, HIV)
Tanda penyakit hati kronis
Splenomegali (infeksi, penyakit myeloproliferatif, limfoproliferatif)
Kulit :
Malar rash (SLE)
Purpura (trombositopenia)
Pigmentasi retikular, kuku displastik (dyskeratosis congenital)
Area hipopigmentasi
Hiperpigmentasi, caf au lait (anemia fanconi
Muskuloskeletal :
Short stature ( fanconi anemia, kelainan congenital lain)
Artritis, sinovitis (SLE)
Jari-jari abnormal (anemia fanconi)
Tanda yang berhubungan dengan infeksi HIV :
Morbiliform rash
Sarkoma kaposi, nodul ulseratif
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LABORATORIUM
Pemeriksaan standar
Hitung darah lengkap
Apusan darah tepi
Hitung retikulosit darah
Fungsi hati dan serologi hepatitis
Tes koagulasi, bleeding time, fibrinogen, D-dimer
Tes direct antiglobulin
Serum B12 dan folat
Seru HIV
TES anti nucleid acid
Biopsi Sumsum Tulang
Selularitas:
: gangguan produksi darah: pasca kemoterapi, sepsis/infeksi akut, HIV Stadium lanjut,
syndrom myelodisplasi hipoplastik, IBMFS,anemia aplastik idiopatik, SLE, PNH
N/: produksi yang tidak efektif/destruksi/sekuestrasi sel-sel darah: myelodisplasia,
leukimia akut/kronik,myeloma dengan plasma selnya, infiltrasi keganasan, HIV stadium
dini/akut, anemia megaloblastik.
Histologi :
Infiltrasi sel
Blast
Gambaran myelodispplasia
Aspirasi Sumsum Tulang
Sitologi (perubahan megaloblastik, perubahan displastik, infiltrasi sel abnormal dan
infeksi)
Imunofenotif (leukimia akut dan kronik, penyakit limfoproliferatif)
Sitogenik (myelodisplasi, leukimia akut dan kronik, penyakit limpoproliferatif).
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Temuan laboratorium
Common
Differential 1 st test Other test
Chemotherapy peripheral blood:anisocytosis,
poikilocytosis, basophilic
stippling
bone marrow aspirate:variable
hypoplasia
bone marrow
biopsy:hypoplasia,
megaloblastosis
Radiotherapy
peripheral blood:anisocytosis,
poikilocytosis, basophilic
stippling
bone marrow aspirate:variable
hypoplasia
bone marrow
biopsy:hypoplasia,
megaloblastosisVitamin B12
deficiency
peripheral blood film:oval
macrocytic RBCs, irregular size
and shape of RBCs (anisocytosis
and poikilocytosis),
hypersegmented granulocytes (>5
lobes)
serum reticulocyte
count:usually low
serum B12:low in B12
deficiency
bone marrow
aspirate:hypercellular,
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megaloblastic erythroblasts, giant
metamyelocytes
serum LDH:moderately raised
serum bilirubin:moderately
raised, mostly indirect
Folic acid
deficiency
peripheral blood film:oval
macrocytic RBCs, irregular size
and shape of RBCs (anisocytosis
and poikilocytosis),
hypersegmented granulocytes (>5
lobes)
serum reticulocyte
count:usually low
serum RBC folate:low in folate
deficiency
bone marrow
aspirate:hypercellular,
megaloblastic erythroblasts, giant
metamyelocytes
serum LDH:moderately raised
serum bilirubin:moderately
raised, mostly indirect
Bone marrowinfiltration by
nonhematologic
malignancy
peripheral blood film:leuko-erythroblastic cell forms
bone marrow aspirate:clumps
of tumor cells
CXR:mass (lung cancer)
CT of abdomen:may revealabdominal or renal mass
serum prostatic-specific
antigen:elevated in prostate
cancer
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serum LFTs:elevated ALT and
AST ( hepatic metastases)
serum coagulation
profile:prolonged PT and PTT
serum fibrinogen and D-
dimer:diminished fibrinogen and
elevated D-dimer (indicative of
chronic disseminated
intravascular coagulation)
thyroid ultrasound:irregular
mass or nodule
breast imaging:mass or
calcifications
Non-Hodgkin
lymphoma
peripheral blood film:variable;
may show circulating lymphoma
cells
bone marrow aspirate:increased
proportion of lymphoid cells
immunophenotyping (of
peripheral blood or bone
marrow):clonal population of
lymphoid cells
lymph nodebiopsy:lymphoproliferative
disorder
Hepatitis C peripheral blood
film:macrocytes, target cells,
stomatocytes, acanthocytes
reticulocyte count:elevated or
normal
serum LFTs:elevated
serum antihepatitis C virus
(HCV):presence of HCV
antibodies
bone marrow
aspirate:hypercellular,
erythroid hyperplasia
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Autoimmune
hepatitis
peripheral blood
film:macrocytes, target cells,
stomatocytes, acanthocytes
reticulocyte count:elevated or
normal
serum LFTs:elevated
autoantibody screen:positive
bone marrow
aspirate:hypercellular,
erythroid hyperplasia
HIV peripheral blood film:atypical
lymphocytes (acute
seroconversion), rouleaux,
dysplastic neutrophils
reticulocyte count:reduced
HIV serology:positive
bone marrow
aspirate:hypercellular (acute
seroconversion), hypocellular,
dyserythropoiesis
protein
electrophoresis:polycl
onal
hypergammaglobulinem
ia
Hepatitis B peripheral blood
film:macrocytes, target cells,stomatocytes, acanthocytes
reticulocyte count:elevated or
normal
serum LFTs:elevated
serum HBsAg:positive
bone marrow
aspirate:hypercellular,erythroid hyperplasia
Hairy cell
leukemia
peripheral blood
film:circulating lymphoma cells
bone marrow aspirate:increased
proportion of lymphoid cells
immunophenotyping (of
peripheral blood or bone
marrow):clonal population of
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lymphoid cells
Chronic
lymphocytic
leukemia
peripheral blood
film:circulating leukemia cells
cells
bone marrow aspirate:increased
proportion of lymphoid cells
immunophenotyping (of
peripheral blood or bone
marrow):clonal population of
lymphoid cells
lymph node
biopsy:lymphoprolifera
tive disorder
Myelodysplasia peripheral blood film:may have
irregular or macrocytic RBCs,
dysplastic granulocytes, platelets
may be large and hypogranular
serum reticulocyte
count:usually low, may be
normal or raised
bone marrow aspirate:usually
hypercellular, rarely, hypocellular
(hypocellular myelodysplasia),
dysplastic changes
cytogenetics:may be
abnormal
Cirrhosis peripheral blood
film:macrocytes, target cells,
stomatocytes, acanthocytes
reticulocyte count:elevated or
bone marrow
aspirate:hypercellular,
erythroid hyperplasia
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normal
serum LFTs:elevated
Cytomegalovirus
infection
peripheral blood film:atypical
lymphocytes, spherocytes if
coexisting hemolysis
CMV-specific IgM and
IgG:positive
bone marrow
aspirate:cellularity usually
increased, hemophagocytosis maybe prominent
bone marrow trephine
biopsy:cellularity usually
increased
Mycobacterial
infection
peripheral blood film:rouleaux
reticulocyte count:reduced
bone marrow aspirate:reduced
cellularity, hemophagocytosis
bone marrow trephine
biopsy:reduced cellularity,
granulomas, fibrosis
bone marrow culture:positive
for organism
Uncommon
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Differential 1sttest Other test
Acute myeloid
leukemia
peripheral blood film:blasts on blood
film, presence of Auer rods
serum PT, PTT, fibrinogen, D-
dimer:may be abnormal, suspect DIC
bone marrow aspiration:usually
hypercellular with blasts, rarely
hypocellular
bone marrow biopsy:presence of
blasts, infiltration, Auer rods
immunophenotyping:detection of
clonal population of blasts
cytogenetics:identification or
nonrandom chromosomal abnormalities
serum PT, PTT, fibrinogen, D-
dimer:prolonged PT, PTT; diminished
fibrinogen, elevated D-dimer
Acute
lymphocyticleukemia
peripheral blood film:blasts may or
may not be present bone marrow aspirate:hypercellular
with blasts; occasionally hypocellular
(childhood ALL)
immunophenotyping (of
peripheral blood or bonemarrow):detection of
clonal population of blasts
cytogenetics:identificatio
n of nonrandom
chromosomal
abnormalities
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Multiple
myeloma
peripheral blood film:rouleaux,
circulating plasma cells may rarely be
present
bone marrow aspirate:plasma cell
infiltrate, abnormal plasma cells,
plasmablasts
immunophenotyping (of peripheral
blood or bone marrow):plasma cells
exhibit restriction of kappa or lambda
light chain expression
serum and urine
electrophoresis:monoclonal serum
protein and urinary Bence-Jones
proteins (light chains) detected
radiologic skeletal
survey:lytic lesions
and/or osteopenia
Myelofibrosis peripheral blood film:leuko-
erythroblastic, tear drop RBCs
bone marrow aspirate:hypercellular
and fibrotic, often dry tap and
nondiagnostic
serum and RBC
folate:usually
diminished
serum B12:usually
elevatedLysosomal
storage
disorders
leukocyte glucocerebroside
activity:reduced or absent
peripheral blood film:pancytopenia
reticulocyte count:may be high,
normal, or reduced
bone marrow aspirate:may reveal
Gaucher cells
bleeding
time:prolonged
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Anorexia
nervosa
peripheral blood film:red cell
acanthocytes, poikilocytosis and
basophilic stippling
reticulocyte count:low
bone marrow aspirate:hypocellular,
reduced hematopoietic cells, may show
gelatinous transformation
bone marrow trephine
biopsy:hypocellular without
infiltration or fibrosis
diepoxybutane test:normal
Graft-versus-
host disease
peripheral blood film:no specific
features
reticulocyte count:low
bone marrow aspirate:hypocellular,
reduced hematopoietic cells, increased
macrophages, erythrophagocytosis
bone marrow trephine
biopsy:hypocellular withoutinfiltration or fibrosis, increased
macrophages
skin, liver, upper GI
biopsy:characteristic appearances of
acute GVHD
HLA typing of peripheral blood
lymphocytes:chimerism
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Heavy metal
(arsenic)
poisoning
peripheral blood film:basophilic
stippling
bone marrow aspirate:hypocellular
without infiltrate or fibrosis, decreased
hematopoietic cells, dyserythropoiesis
bone marrow trephine
biopsy:hypocellular without
infiltration or fibrosis dyserythropoiesis
diepoxybutane test:normal
screening for PNH clone:negative
arsenic level (serum, urine, hair,
nails):elevated
Parvovirus
infection in
sickle cell
anemia
CBC:drop in hemoglobin
concentration of >30 percent secondary
to complete arrest of erythropoiesis
reticulocyte count:decrease or
absence of measurable reticulocytes
bone marrow biopsy:remarkable for
severe aplasia
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Dyskeratosis
congenita
peripheral blood film:red cells
usually macrocytic
reticulocyte count:low or absent
bone marrow aspirate:hypocellular,
reduced hematopoietic cells,
dyserythropoiesis common
bone marrow trephine
biopsy:hypocellular without
infiltration or fibrosis
diepoxybutane test:normal
(peripheral blood lymphocytes)
screening for PNH clone:may be
present
peripheral blood and/or bone
marrow
immunophenotyping:normal
blood and/or bone marrow
cytogenetics:clonal abnormalities
present in some patients
genetic studies:may
identify 1 of several
genetic mutations
telomere
length:abnormally
short; length in
lymphocytes and
granulocytes
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Idiopathic
aplastic
anemia
peripheral blood film:normocytic or
mildly macrocytic RBCs, no immature
precursors present
serum reticulocyte count:low or
absent
bone marrow aspirate:hypocellular,
mild dyserythropoiesis common
bone marrow trephine
biopsy:hypocellular without fibrosis
or infiltrate
diepoxybutane test (peripheral blood
lymphocytes):normal
screening for paroxysmal nocturnal
hemoglobinuria clone (peripheral
blood, bone marrow):detectable in up
to 30% of patients
peripheral blood, bone marrow
immunophenotyping:normal
peripheral blood, bone marrowcytogenetics:abnormal clones present
in a minority of patients
Fanconi
anemia
peripheral blood film:RBCs usually
macrocytic
reticulocyte count:low or absent
bone marrow aspirate:hypocellular
dyserythropoiesis
diepoxybutane test (peripheral blood
lymphocytes):increased chromosomal
breakage
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Idiopathic
portal
hypertension
peripheral blood film:no specific
features
reticulocyte count:elevated or normal
serum LFTs:normal or mildly
elevated
bone marrow
aspirate:hypercellular
, erythroid hyperplasia
bone marrow
trephine
biopsy:hypercellular,
erythroid hyperplasia
Chronic
myeloid
leukemia
peripheral blood film:myeloid
maturing cells, elevated basophils,
eosinophils
cytogenetics:Philadelphia
chromosome positive
bone marrow biopsy:granulocytic
hyperplasia
Brucellosis peripheral blood film:no specific
features
bone marrow aspirate:trilineage
hypercellularity, hematophagocytosis
blood and bone marrowcultures:positive for organism
Leishmaniasis peripheral blood film:rouleaux;
organisms rarely seen in peripheral
blood film
bone marrow aspirate:trilineage
hypercellularity; organisms may be
seen within macrophages (Leishman-
Donovan bodies)
bone marrow trephine
biopsy:trilineage hypercellularity,
hematophagocytosis, small
granulomata
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immunochromatographic or PCR-
based tests on peripheral blood or
bone marrow aspirate:positive for
organism
Hemophagocy
tosis
syndromes
peripheral blood film:no specific
features
bone marrow aspirate:trilineage
hypercellularity, hematophagocytosis
blood and bone marrow
cultures:positive for organism
autoimmune
screen:positive ANA and
anti-ds DNA
serum ferritin:>10,000
mcg/L
molecular genetic
testing:specific karyotype
present
Drug-induced
immune
pancytopenia
platelet-specific antibodies:positive
peripheral blood film:no specific
features
reticulocyte count:elevated
bone marrow aspirate:hypercellular
bone marrow trephine
biopsy:hypercellular
Evanssyndrome
with
associated
neutropenia
peripheral blood film:polychromasia,spherocytes
reticulocyte count:elevated
direct antiglobulin test:positive
platelet, neutrophil-specific
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antibodies:positive
bone marrow aspirate:normal or
trilineage hypercellularity
bone marrow trephine
biopsy:normal or trilineage
hypercellularity
Systemic
lupus
erythematosus
peripheral blood film:rouleaux
autoimmune screen:positive ANA
and anti-DNA
ultrasound of the
abdomen:splenomegaly
bone marrow aspirate:hypocellular,
Adysplastic changes,
hematophagocytosis
bone marrow trephine
biopsy:hypocellular, benign lymphoid
aggregates
Rheumatoid
arthritis
peripheral blood film:rouleaux
autoimmune screen:positiverheumatoid factor
bone marrow aspirate:hypocellular,
dysplastic changes,
hematophagocytosis
bone marrow trephine
biopsy:hypocellular, benign lymphoid
aggregates
ultrasound of the
abdomen:splenomegaly
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Infectious
mononucleosi
s
serum monospot:positive
peripheral blood film:atypical
lymphocytes
Epstein-Barr nuclear
antibody:present
blood serology (specific IgM and IgG
titers) for viral capsid
antigen:positive
Felty
syndrome
bone marrow biopsy:myeloid
hyperplasia with excess of immature
forms
autoimmune screen:positive
rheumatoid factor
ultrasound of the
abdomen:splenomegal
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BAB III
Kesimpulan
Pansitopenia bukanlah suatu gejala melainkan suatu triad yang di sebabkan suatu proses penyakit
yang melibatkan sumsum tulang baik primer maupun sekunder. Pansitopenia adalah masalah
sering di temui dalam praktek sehari-hari. Untuk menegakan diagnosis pati perlu di lakukan
pemeriksaan yang lebih spesifik.
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Tinjauan Pustaka
1. Evaluation of pancytopenia Diagnostic approach-Eppocrates online
hhtps://online.epocrates.com/u/29211024/evaluation+of+pancytopenia.
2. Makalah lengkap Bandung hematologi oncology meeting 2013, page 7-15