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
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