Aplastic Anaemia

1. Primary idiopathic acquired aplastic anaemia

2. Secondary aplastic anaemia

Primary idiopathoc acquired aplastic anaemia

The basic problem is failure of pluripotent stem cells, producing hypoplasia of the bone marrow with a pancytopenia in the blood.

Clinical features and investigations

• Patients present with symptoms of bone marrow failure, usually anaemia or bleeding and less commonly infections.

• FBC shows pancytopenia, low reticulocytes and often macrocytosis.

•Bone marrow aspiration and trephine reveal hypocellularity.

Management

All patients will require blood product support and aggressive management of infection.

The curative treatment is allogeneic HSCT if there is available donor.

In older patients, immunosuppressive therapy with CICLOSPORIN and ANTITHYMOCYTE GLOBULIN gives 5-year survival rates of 75%.

Secondary aplastic anaemia

Clinical features and methods of diagnosis are same as for primary idiopathic aplastic anaemia.

Causes

• Drugs

Cytotoxic drugs

Antibiotics - chloramphenicol, sulphonamides

Antirheumatic agents - penacillamine, gold, phenylbutazone, indometacin

Antithyroid drugs

Anticonvulsants

Immunosuppressants - azathioprine

•Chemicals

Benzene toluene solvent misuse

Insecticides - DDT

Organophosphates and carbamates

• Radiation

• Viral hepatitis

• Pregnancy

• paroxysmal nocturnal haemaglobinuria

Comments

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