Pathology
Bonemarrow aplasia leading to pancytopenia.
Aetiology
- Idiopathic – Most cases
- Hereditary – Fanconi Syndrome
- Post Viral – Hepatitis A, B, C
- Drug induced – Carbamazepine, busulfan
- Radiation – Exposure to high doses of radiation
Symptoms
Shortness of breath, palpitations, chest pain, bleeding, easy bruising, recurrent infections.
Signs
Pallor, bruising, increased respiratory rate.
Investigations
- Bloods: FBC –pancytopenia
- BloodFilm: Reduction in number of visible cells
- Bone Marrow Aspirate: Hypocellular Marrow
Treatment
Careful observation, blood transfusion, immunosuppressive therapy, and bone marrow transplant in severe cases.
Complications
- Progression to more severe disease, progression to acute leukaemia.
- Development of paroxysmal nocturnal haemoglobinuria.
Prognosis
Potential for cure with bone marrow transplant, immunosuppression may give good results.
