(13) Aplastic Anemia

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    APLASTIK ANEMIA

    Aplasia of bone marrow:quantity or function :

    Hemopoietic tissue (blood forming-elements)

    decrease activity of bone marrow

    adult blood cell forming

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    Aplastik anemia

    pancytopenia

    Pancytopenia:

    All of hemopoetic cells system

    - anemia- leucopenia- thrombocytopenia

    By various causes

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    PATOFISIOLOGI

    Basic disorder: disturbance/damage of:1. Plurypotent stem cell :

    - quantity & function decrease

    proliferation & differentiation

    2. Microenvironment (marrow environment):

    - Microvaskular disorder

    - Abnormality of humoral factor- Inhibition substance of growth cell

    bone marrow tissue unable togrowth and develop

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    CAUSE FACTOR :

    I. Primary/Idiopathic

    50%

    II. Secondary:

    1. Drugs2. Chemical3. Radiation4. Infection5. Constitutional/familial

    1. Drugs- structure: hydrocarbon benzene- dose & duration of consumption

    - individual susceptible

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    Drugs bone marrow depression:

    a. Often/always depress bone marrow:

    - Cytostatic

    b. Sometimes depress bone marrow:

    - Antibiotic: chloramfenikol, sulfonamide ect

    - Anticonvulsant: methylhidantoin- Analgesic: Phenylbutasone- Muscle relaxant : meprobamate

    2. Chemical

    - inhalation, skin contact & oral (accident)

    insecticide, organic dilution, ect

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    3. Radiation

    fast growth tissue:

    - hemopoesis tissue- gonad germinal cell- epithel of intestine

    Radiation cause damage of:

    - stem cell critical period 3-6 week- stem cell environment capillary

    4. Infection

    - immunologic mechanism- cause agent stem cell

    5. Constitutional /familial/ congenital

    Fanconi Syndrome

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    MANIFESTATION/SIMPTOMS

    The onset usually gradually

    1. Clinical symptoms Pancytopenia (important sign):

    - anemia general symptoms of anemia

    - granulocytopenia: easy to get infection

    - thrombocytopenia: bleeding

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    2. Peripheral blood:

    - normochrome normocytic anemia

    - low reticulocyte(normal: 50.000-100.000/mm3)

    - relative lymphocytosis

    3. Bone marrow:

    - hypocellular (bone marrow aplasia)

    relative frequently: lymphoid cell& reticulum

    - many adipose tissueBone marrow puncture is very important !

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    5. Hb F can increased

    6. Fe serum

    DIFFERENTIAL DIAGNOSIS

    1. ITP and ATP2. Leukemia aleukemic

    pancytopenia

    reticulocytopenirelative lymphocytosis

    Blast cell or lymphocytosis > 90 % ALL

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    TREATMENT

    Principal of treatment:

    - Remove the causes

    - Avoid trauma mucosa & skin- Avoid infection

    - Bone marrow stimulation (hemopoesis)

    androgen hormone: testosterone & oximetolon

    - Blood transfusion:

    as minimum as possible Hb 8 - 9 g/dl

    - fraction: PRC, trombocyte & granulocyte

    suspension

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    - Replacement the damage stem cell

    bone marrow transplantation

    - Other treatment:

    Corticosteroid severe thrombocytopenia

    Splenectomi resistance cases

    Immunosuppressive agentimmunological cause

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    PROGNOSIS

    - Age

    - Bone marrow picture

    - hypocellular- acellular

    - HbF concentration>200 mg%: good prognosis

    - Granulocyte:> 2000/mm3: good prognosis

    - Infection

    Cause of death:

    - Infection

    - Bleeding