Secondary acquired pure red cell aplasia

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Pure red cell aplasia is a well known association with thymoma. About 5% of thymomas are associated with PRCA and about 10-20% of patients with PRCA have a thymoma. The majority of tumours show spindle cell morphology but some are

Table 13.5 Clinical classification ofpure red cell aplasia.

Congenital

Diamond-Blackfan anaemia

Acquired paediatric

Transient erythroblastopenia of childhood Parvovirus-induced aplastic crisis

Systemic lupus erythematosus, rheumatoid arthritis Chronic lymphocytic leukaemia, lymphoma

Primary autoimmune Secondary

T-large granular lymphocytosis Thymoma

Myelodysplastic syndrome Hypogammaglobulinaemia

Drugs: azathioprine, isoniazid, phenytoin, rHuEPO Hepatitis, Epstein-Barr virus, parvovirus Others: major ABO mis-match BMT, pregnancy

lymphomas. The tumour may usually be diagnosed on plain radiography but tomography or computerized tomography scanning may be required. There may be associated abnormalities of other systems linked to the thymoma, for example myasthenia gravis. The response to thymectomy is unpredictable. About one-half of the patients respond to surgery but some relapse later. Some patients develop PRCA only after removal of the thymoma and some show no response. For patients who fail to respond to thymectomy, immunosuppressive treatment is used (see below).

Pure red cell aplasia may be associated with a variety of lymphoid disorders including B- and T-cell lymphomas and particularly T-large granular lymphocytosis. The main conditions are shown in Table 13.5. The PRCA may precede the appearance of the lymphoma but the latter may be identified in some patients at the time of diagnosis of the PRCA by immunoglobulin or T-cell receptor gene rearrangement studies. The anaemia usually, but not always, responds to treatment of the lymphoma.

Pure red cell aplasia is associated with a number of autoimmune disorders. There may be a positive direct antiglobulin test (DAT) that indicates the nature of the aplasia. It occurs in patients with agammaglobulinaemia, and may also be associated with systemic lupus erythematosus, Sjogren's syndrome or rheumatoid arthritis. The majority of these anaemias will respond to treatment with corticosteroids, with or without azathioprine. The response may be steroid-dependent or there may be a relapsing and remitting course.

A long list of drugs have been incriminated in the aetiology of PRCA, but the association is very rare and mostly there is only a single case for each drug. Exceptions are azathioprine, phenytoin, procainamide and isoniazid, for which several cases each have been reported. More recently, recombinant human erythropoietin (rHuEpo), when used predominantly in chronic renal failure patients, has been reported to cause PRCA associated with anti-erythropoietin antibodies, resulting in a sudden and severe anaemia that is unresponsive to increased dose of rHuEpo. In this situation the rHuEpo must be discontinued immediately and regular blood transfusional support given; immunosuppressive therapy may be required for the PRCA.

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