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APLASTIC ANEMIA

Dr H IRZA WAHID SpPD KHOM FINASIM


SUBDIVISION OF HEMATOLOGY & MEDICAL ONCOLOGY
DEPARTEMENT OF INTERNAL MEDICINE
FACULTY OF MEDICINE ANDALAS UNIVERSITY
Hematologi Dan Onkologi Medik
Hematologi : Ilmu + Darah

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

* Eritropoesis eritrosit Anemia / Polisitemia


* Megakariopoesis trombosit Trombositosis / Trombositopenia
* Granulopoesis leukosit Leukopenia / Leukositosis
* Monositopoesis monosit Monositopenia / Monositosis
* Limfopoesis limfosit Limfositopenia / Limfositosis

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

Onkologi Medik : Ilmu + Tumor + Non Surgery


ANEMIA
PARAMETER : Kadar hemoglobin Metode Sahli
Pria dewasa : Wanita dewasa : Hamil : Hb < 13 : < 12 : < 11 gr %

GEJALA & TANDA


Hb hipoksia kompensasi kardiovaskular
* Pucat * angina pektoris * kardiomegali
Mukosa * claudicatio intermiten * palpitasi
Kulit * tinitus * dispneu
* berkunang * bising sistolik
* cepat lelah * gagal jantung
DIAGNOSIS
Gradasi anemia 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
Definition
Pancytopenia
Anemia
Neutropenia
Thrombocytopenia

Reticulocyto-penia

Aplastic bone marrow


Hypocellular with all
elements down; mostly fat
and stroma
Residual hematopoietic
cells are normal
No malignancy or fibrosis
No megaloblastic
hematopoiesis
Severity of Disease
Severe Aplastic Anemia (SAA)
Marrow of less than 25% normal cellularity
2 of 3 abnormal peripheral blood values
Absolute reticulocyte count < 40,000
ANC<500
Platelets <20,000

Very Severe Aplastic Anemia (VSAA)


Above with ANC <200
Etiology
Inherited
Fanconis Anemia

Acquired
Idiopathic (Primary) 2/3 of cases
Drug : Acetazolamide, Carbamazepine, Gold,
Hydantoin, Penicillin, Phenylbutazone,
chloramphenicol
Chemical
Radiation exposure
Viral illness
Pathogenesis

Immune mechanism responsible for


most of the cases of Idiopathic
acquired aplastic anemia
Activated Cytotoxic T cells in Blood
& Bone marrow Bone marrow
failure
Clinical Features

Anemia

Bleeding: Ecchymoses ,Bleeding


gums, Epistaxis

Infections: Fever,Mouth ulcers


Diagnosis

Blood peripheral smear :


Pancytopenia and reticulocytopenia

Bone marrow aspiration & biopsy :


Hypocellular / aplastic bone marrow
with increased fat spaces

Tests for underlying cause ( viral


titers)
BM Aspiration BM Biopsy
BM biopsy
hypocellular ,increased fat spaces
Other causes of Pancytopenia:
Drugs,
Megaloblastic anemia
Bone Marrow infiltration or Replacement:
Lymphoma, Myeloma,Acute Leukemia
Hyperspleenisn
SLE
Disseminated TB
PNH
Sepsis
Definitive therapy:
Immunosuppression
Immunosuppression is NOT curative
Goal is sustained remission
20-36% have recurrent aplastic anemia
20-36% develop clonal disorder, PNH, MDS or acute
leukemia
Combination therapy is best
Antithymocyte globulin (ATG) first choice
Toxic side effect is serum sickness, tx with steroid
Can lower platelet counts, transfuse prn
Cyclosporine first choice
High dose corticosteroids
Alemtuzumab