APLASTIK ANEMIA

Aplasia of bone marrow:

quantity or function :
Hemopoietic tissue (blood forming-elements)
decrease activity of bone marrow
adult blood cell forming
Aplastik anemia pancytopenia
Pancytopenia:
All of hemopoetic cells system
- anemia
- leucopenia
- thrombocytopenia
By various causes
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 to
growth and develop
 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
 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
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
 MANIFESTATION/SIMPTOMS
The onset usually gradually

1. Clinical symptoms Pancytopenia (important sign):

- anemia general symptoms of anemia
- granulocytopenia: easy to get infection
- 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

- Bone marrow stimulation (hemopoesis)

androgen hormone: testosterone & oximetolone

- Blood transfusion:
as minimum as possible Hb 8 - 9 g/dl
- fraction: PRC, trombocyte & granulocyte
suspension
- Replacement the damage stem cell
bone marrow transplantation

- Other treatment:
Corticosteroid severe thrombocytopenia
Splenectomi resistance cases
Immunosuppressive agent
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