Aplastic Anaemia

Clinical Description

It is an abnormality in at least 2 blood cell lines with hypocellular bone marrow.

Causes

Primary causes: Inherited (e.g., Fanconi anaemia) or idiopathic (acquired and accounts about 67% of aplastic anaemia cases)

Secondary causes: Chemicals (e.g., benzene, toluene, glue sniffing), drugs (e.g., chemotherapeutic drugs, chloramphenicol, gold, penicillamine, phenytoin, carbamazepine, azathioprine), insecticides, ionizing radiation, infections (e.g., HIV, EBV, Viral hepatitis, TB, Parvovirus B19), paroxysmal nocturnal haemoglobinuria and pregnancy.

Signs and Symptoms

• Easy bruising, bleeding, blood blisters in the mouth
• Pallor
• Petechiae
• Purpura
• Bleeding
• Frequent or severe infections

Investigations

• FBC: Pancytopenia
• PBF: Reduced blood cells and absence of reticulocytes
• Bone marrow aspiration and trephine: hypocellular bone marrow
• Other investigations: to exclude secondary causes (e.g., HIV, TB screen, Hepatitis B and C serology)
• Refer to central hospital for further workup

Treatment

• Treatment is largely supportive
• Treat infections aggressively
• If anaemic and/or bleeding, transfuse whole blood and/or platelets
• If neutropenic and febrile: antibiotics

Complications and Referral Criteria

• Discuss all cases of suspected aplastic anaemia with a haematologist/specialist.
• Stabilise the patient, if necessary, with blood products before referral.