Primary digestive Richter's syndrome

The clinical and morphologic transformation of 3 to 5% of chronic lymphocyt ic leukemia (CLL) to diffuse large-cell lymphoma (DICL) is commonly referre d to as Richter's syndrome. Richter's syndrome occurs mostly in lymph nodes and may represent a second neoplasm or a transformation from the same clon al population. Clinical features in six patients with digestive Richter's s yndrome were recorded. Paired samples of CLL and DLCL were investigated by immunohistological analysis (n = 6) and by polymerase chain reaction (PCR) for immunoglobulin heavy-chain gene rearrangement (n = 4). Histological exa mination revealed the involvement of the gastrointestinal tract by DLCL of B-cell phenotype (n = 6). The same monoclonal rearrangement between CLL and DLCL was demonstrated by PCR and sequencing analyses in two patients. The monoclonal rearrangement was different between CLL and DLCL in only one cas e. Median survival was 22 months for five patients receiving chemotherapy, suggesting that digestive Richter's syndrome has a better prognosis than no dal Richter's syndrome. Indeed, appropriate surgical resection combined wit h chemotherapy led to partial or complete remission in four patients.