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    Anemia Aplastik

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    Panca Sapriawan
    Aplastic anemia is a bone marrow failure syndrome characterized by pancytopenia and a hypocellular bone marrow. It results from immune-mediated destruction of hematopoietic stem cells. Symptoms include anemia, bleeding, and infections due to low blood cell counts. Diagnosis involves blood tests showing pancytopenia and a bone marrow aspiration demonstrating a hypocellular marrow with fat cells and few remaining hematopoietic cells. Definitive treatment is immunosuppression with antithymocyte globulin and cyclosporine to suppress the immune system and allow hematopoietic recovery.

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    Anemia Aplastik

    Uploaded by

    Panca Sapriawan
    130 views18 pages
    Aplastic anemia is a bone marrow failure syndrome characterized by pancytopenia and a hypocellular bone marrow. It results from immune-mediated destruction of hematopoietic stem cells. Symptoms include anemia, bleeding, and infections due to low blood cell counts. Diagnosis involves blood tests showing pancytopenia and a bone marrow aspiration demonstrating a hypocellular marrow with fat cells and few remaining hematopoietic cells. Definitive treatment is immunosuppression with antithymocyte globulin and cyclosporine to suppress the immune system and allow hematopoietic recovery.

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    hematologi

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    Aplastic anemia is a bone marrow failure syndrome characterized by pancytopenia and a hypocellular bone marrow. It results from immune-mediated destruction of hematopoietic stem cells. Symptoms include anemia, bleeding, and infections due to low blood cell counts. Diagnosis involves blood tests showing pancytopenia and a bone marrow aspiration demonstrating a hypocellular marrow with fat cells and few remaining hematopoietic cells. Definitive treatment is immunosuppression with antithymocyte globulin and cyclosporine to suppress the immune system and allow hematopoietic recovery.

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    © All Rights Reserved
    Download as PPT, PDF, TXT or read online from Scribd
    Download as ppt, pdf, or txt
    130 views18 pages

    Anemia Aplastik

    Uploaded by

    Panca Sapriawan
    Aplastic anemia is a bone marrow failure syndrome characterized by pancytopenia and a hypocellular bone marrow. It results from immune-mediated destruction of hematopoietic stem cells. Symptoms include anemia, bleeding, and infections due to low blood cell counts. Diagnosis involves blood tests showing pancytopenia and a bone marrow aspiration demonstrating a hypocellular marrow with fat cells and few remaining hematopoietic cells. Definitive treatment is immunosuppression with antithymocyte globulin and cyclosporine to suppress the immune system and allow hematopoietic recovery.

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    © All Rights Reserved
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    of 18

    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

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