Biology and treatment of richter syndrome blood american. The reported incidence of transformation to rt in patients with cll is around 4% and the risk of rt is independent of disease stage, duration, or response to prior treatment. How we treat richter syndrome blood american society. How we treat richter syndrome blood american society of. Autologous and allogeneic stemcell transplantation for transformed chronic lymphocytic leukemia richters syndrome. About 1% of cll cases annually transform to a more aggressive lymphoma. Mar, 2014 each year approximately 16 000 individuals in the united states are diagnosed with cll. Ibrutinib and rituximab induced rapid response in refractory. Richters syndrome rs, also known as richters transformation, is a transformation of b cell.
Abstract richter s syndrome denotes the leukemic evolution to highgrade nonhodgkins lymphoma, prolymphocytic leukemia, hodgkins. Over time, a fraction of cll patients develop an aggressive largecell lymphoma called richter s syndrome rs or richter s transformation rt. Abstract richters syndrome denotes the leukemic evolution to highgrade nonhodgkins lymphoma, prolymphocytic leukemia, hodgkins. Jun 05, 2015 richter syndrome describes the transformation of cll into an aggressive lymphoma. Richter syndrome rs consists of the transformation of chronic lymphocytic leukemia cll to diffuse large bcell lymphoma dlbcl, a rapidly growing variety of nonhodgkins lymphoma with poor prognosis. Rs refers to the development of highgrade nonhodgkin lymphoma in a person who has chronic lymphocytic leukemia cllsmall lymphocytic lymphoma sll. Richter s transformation, or richter s syndrome, is an uncommon clinicopathological condition observed in about 5% to 10% of patients with chronic lymphocytic leukemia cll. Richter s syndrome rs, also known as richter s transformation, is a transformation of b cell chronic lymphocytic leukemia cll or hairy cell leukemia into a fastgrowing diffuse large b cell lymphoma, a variety of nonhodgkin lymphoma which is refractory to treatment and carries a bad prognosis. Il linfoma e spesso di tipo b, raramente di tipo t. Richters syndrome rs, also known as richters transformation, refers to the transformation of one specific blood cancer type into a different, more aggressive type. Richter syndrome with extensive isolated pleural extranodal.
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