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What is the treatment for Evans syndrome?

Q: I am suffering from a disease called Evans syndrome (anaemia). My platelet count had gone under 30,000 and my RBCs were around 5. I was given some steroid treatment, which helped me gain my RBCs and platelet count. I have been taking these steroids for the last 5 weeks now. The doctor has said that if I do not get the required level in 2 weeks, he will have to remove my spleen. Is there no way to cure it without removing spleen?

A:Evans syndrome is a condition in which antibodies are formed in the body directed against red blood cells (causing their breakdown leading to anaemia), platelets (causing low platelet counts) and sometimes also the white blood cells. Its cause is unknown but is thought to be some disorder in a type of white blood cells of the body called lymphocytes, which triggers antibody formation. The disease waxes and wanes and often responds poorly to treatment. The patients have recurrence of anaemia (low haemoglobin), thrombocytopaenia (low platelets) and neutropaenia (low neutrophils) predisposing them to bleeding and infections. Currently, patients are treated with steroids like prednisolone and once the acute phase is over, an attempt is made to wean them off the drug. In case of refractoriness, intravenous gammaglobulin (IVIG) is used, though it is more effective in treating the low platelet counts than haemolysis. The drug can be used intermittently. Other treatment modalities include the use of immunomodulating drugs like danazol, cyclosporine, azathioprine, cyclophosphamide and vincristine. A new drug which has been tried is rituximab but with mixed results. The removal of spleen (splenectomy) is done when medical therapy fails. It may improve blood counts and reduce steroid dependence, but relapses are common and its effects may last for only a week or so, up to five years.


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