Patients with B-chronic lymphocytic leukaemia /small lymphocytic lymphoma (CLL) have a 5-10% risk of developing autoimmune complications, which primarily cause cytopaenia. These autoimmune cytopaenias can occur at any stage of CLL and do not have independent prognostic significance. The most common autoimmune complication is autoimmune haemolytic anaemia with a lower frequency of immune thrombocytopaenia and pure red blood cell aplasia and only rarely, autoimmune granulocytopaenia (AIG). Autoimmune cytopaenia should always be considered in the differential diagnosis of cytopaenia in patients with CLL. Patients with CLL can also have more than one form of autoimmune cytopaenia, which can occur together with bone-marrow failure. Treatment is usually effective but rarely curative for autoimmune cytopaenia complicating CLL. Optimal therapy will depend on a timely and accurate diagnosis of autoimmune cytopaenia and should be individualised according to the severity of the cytopaenia and the presence or absence of concomitant progressive CLL requiring therapy.
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