Sequential lymphomas or clonally unrelated richter syndrome of chronic lymphocytic leukemia into mantle cell lymphoma.

Abstract

Clinical Practice Points Patients with chronic lymphocytic leukemia (CLL) can present with transformation into aggressive lymphomas (Richter’s transformation), most commonly diffuse large B cell lymphoma (DLBCL). However, other types of lymphomas can also occur in patients with CLL, e.g. the present case. A clinical suspicion of Richter syndrome by clinical history, abnormal laboratory, and positron emission imaging findings must be followed by a proper biopsy (not fine needle aspirate) and detailed histopathologic evaluation of the lymph nodes or involved tissues to confirm the diagnosis of the type of transformation or the type of lymphoma. Not all clinically suspected Richter syndrome cases are diffuse large B cell lymphoma. Simultaneous presentation of mantle cell lymphoma (MCL) and CLL in the same lymph node or in the same patient at the same time has been well described. We describe the unusual case of a patient with CLL who was in complete remission from CLL and now presented with a MCL without any evidence of CLL, which suggests a sequential lymphoma or a transformation of CLL into MCL.