(13) aplastic anemia
TRANSCRIPT
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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