Image Description
In about 5% to 10% of patients with CLL/SLL, the disease undergoes transformation to a more aggressive tumor, usually in the form of a diffuse large B-cell lymphoma (shown in this image). This phenomenon has been termed Richter Syndrome. The first manifestations are usually seen in extramedullary sites. for e.g. a rapidly enlarging mass may develop within the involved lymph node or spleen. The prognosis in this event is extremely poor with life expectancy of less than 1 year in most cases. An increased incidence of Richter syndrome has been noticed in CLL patients treated with Fludarabine. It occurs within the first year of therapy and is related to Epstein-Barr virus.
About the Disease
B-cell chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) are the most common mature B-cell neoplasms and considered to be different manifestations of the same disease. CLL: It is the most common mature B-cell leukemia in the Western hemisphere and makes up 30% of all leukemias. It mainly affects middle-aged and elderly patients with a male predominance. The neoplastic cells are predominantly in blood and bone marrow. CLL is asymptomatic in most patients and often detected incidentally on complete blood counts. The diagnosis requires absolute mature lymphocytosis of ≥ 5.0 x 109/L sustained for at least 3 months. Immunophenotype: Multicolor flow cytometry shows the following results in a typical case: mature B-cell phenotype with weak monotypic surface immunoglobulin, CD5 coexpression, weak CD20, weak CD22, CD23, CD19, weak CD11c; negative for CD10, FMC7, and CD79b. SLL: In contrast to CLL, SLL is much less common, making up only about 4% of non-Hodgkin lymphomas. The distribution of the neoplastic cells in SLL is mainly extramedullary with very few leukemic cells in blood. The patients are often asymptomatic but may present with lymphadenopathy and splenomegaly.