(13) aplastic anemia

8/10/2019 (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

(13) aplastic anemia

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