Marrow Failure - Aplastic Anaemia

Features:
- Pancytopenia
- Hypoplastic marrow
- Low reticulocyte count

Si&Sy
Infection/bleeding/anaemia

Causes:
Congenital
Fanconi's (not*)
Acquired
Idiopathic - *AI reaction to BM Stem Cells - eg T suppressor lymphocytes
Radiation - RTx/Occ
Chem - Benzene TNT, DDT
Drugs - Cytotoxic Dx (requires frequent FBCs), other
Viruses

Viral Causes

 - Hepatitis

 - EBV

 - ParvoV

Non-cytotoxic Drug Reactions

 - Chloamphenicol

 - Zidovudine

 - Gold

 - Phenylbutazone

Tx

Remove cause

Supportive - red cell transfusion, ABs, plt transfusion

Stim BM Stem Cells - anabolic steroids

Severe AA
- Np < 0.5
- Plts < 20
- Transfusion-dependant Hb

Tx
BM transplant
- <45yrs
- with HLA matched donor avail
ATG/ALG (anti-thymocyte globulin)
 - removes inhibitory T-cells
 - H/e SE: temp thrombocytopenia & serum sickness
Immsupp
- cyclosporin
- HD steroids