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By various causes
PATOFISIOLOGI
Basic disorder: disturbance/damage of:
1. Plurypotent stem cell :
- quantity & function decrease
proliferation & differentiation
CAUSE FACTOR :
I. Primary/Idiopathic
50%
II. Secondary:
1. Drugs
2. Chemical
3. Radiation
4. Infection
5. Constitutional/familial
1. Drugs
- structure: hydrocarbon benzene
- dose & duration of consumption
- individual susceptible
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
MANIFESTATION/SIMPTOMS
The onset usually gradually
- thrombocytopenia: bleeding
<50.000/mm3: spontaneous bleeding
<20.000/mm3: cerebral and gastrointestinal
bleeding
prolonged bleeding time
No RES organ enlargement
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 tissue
Bone marrow puncture is very important !
5. Hb F can increased
6. Fe serum
DIFFERENTIAL DIAGNOSIS
1. ITP and ATP
2. Leukemia aleukemic
pancytopenia
reticulocytopeni
relative lymphocytosis
Blast cell or lymphocytosis > 90 % ALL
TREATMENT
Principal of treatment:
- Remove the causes
- Avoid trauma mucosa & skin
- Avoid infection
immunological cause
PROGNOSIS
- Age
- Bone marrow picture
- hypocellular
- acellular
- HbF concentration
>200 mg%: good prognosis
- Granulocyte:
> 2000/mm3: good prognosis
- Infection
Cause of death:
- Infection
- Bleeding