Although rare cases of prolymphocytic transformation from splenic
B-cell lymphomas and
follicular lymphoma have been reported, prolymphocytic transformation from lymphoplasmacytic
lymphoma has not been previously reported. We report a case of 76-year-old-male patient with a history of Waldenström
macroglobulinemia diagnosed in 2010 and treated with infusion
chemotherapy. He was in clinical remission for 5 years. In 2016, he presented with diffuse
lymphadenopathy, and a head and neck lymph node biopsy showed lymphoplasmacytic
lymphoma. MYD88 mutation was detected by polymerase chain reaction. A subsequent bone marrow biopsy showed
B-cell lymphoma with increased prolymphocytes. Peripheral blood showed numerous circulating prolymphocytes. MYD88 was detected by polymerase chain reaction in the bone marrow. Cerebrospinal fluid was positive for
lymphoma cells with prolymphocytic morphology. An
IgM κ
paraprotein was noted by immunofixation performed on the patient's serum, urine, and cerebrospinal fluid. The patient was resistant to
chemotherapy, developed multiorgan failure, and died shortly thereafter.