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294 / Chapter 22 Aplastic anaemia and bone marrow failure

mismatched family members is achieving a sur- number of somatic disorders (e.g. of the face or
vival ratio over 50% in patients with severe aplas- heart). Mutation of a gene on chromosome 19 or
tic anaemia. In older subjects and those with less other genes that encode ribosomal proteins under-
severe disease, immunosuppression is usually lies most cases (Fig. 22.3 ). Corticosteriods are the

tried fi rst. first line of treatment and SCT may be curative.
6 Haemopoietic growth factors Granulocyte col- Androgens may also produce improvement but
ony - stimulating factor (G - CSF) may produce side - eff ects on growth can be severe.

minor responses but does not lead to sustained The acquired chronic form can occur without
improvement. Other growth factors have not any obvious associated disease or precipitating
proved helpful. factor (idiopathic), or may be seen with autoim-
mune diseases (especially systemic lupus erythema-
tosus), with a thymoma, lymphoma or chronic
Red c ell a plasia
lymphocytic leukaemia (CLL). Red cell aplasia
from anti - erythropoietin antibodies has been rarely
Chronic
described in patients with chronic renal failure

This is a rare syndrome characterized by anaemia receiving recombinant erythropoietin. In some
with normal leucocytes and platelets and grossly cases, immunosuppression with corticosteroids,
reduced or absent erythroblasts in the marrow (Fig. ciclosporin, azathioprine or ALG is helpful.

22.3 ). The congenital form is known as Diamond – Thymectomy may help in those with a thymoma,
Blackfan syndrome (Table 22.3 ) and is inherited as and treatment of the underlying disease may help
a recessive condition. It is associated with a varying cases secondary to lymphoma or CLL. Monoclonal

Table 22.3 Classifi cation of pure red cell aplasia.

Acute, transient Chronic congenital Chronic acquired
Parvovirus infection Diamond – Blackfan Idiopathic
Drugs (e.g. azathioprine, syndrome Associated with thymoma, systemic lupus
co - trimoxazole) erythematosus, rheumatoid arthritis, lymphoma,
Idiopathic in infancy and chronic lymphocytic leukaemia, T - large granular
childhood lymphocytosis, myelodysplasia, viral infection,
drugs

Figure 22.4 The bone marrow in
primary red cell aplasia. There is
selective loss of erythropoiesis.