Immunoblastic sarcoma following Waldenström's macroglobulinemia

Immunoblastic sarcoma has been observed in association with, or subsequent to, chronic immune stimulation, connective tissue disorders, and immunoblastic lymphadenopathy. A case of Waldenström's macroglobulinemia progressinng after a few years into immunoblastic sarcoma is reported. Splenectomy led to disappearance of hemolytic anemia and of pulmonary infiltrates, as well as to marked reduction of macroglobulins. Although immunoblastic sarcoma usually terminates fatally within two or three months, complete remission was induced by combination chemotherapy. It is speculated that Waldenström's macroglobulinemia, immunoblastic lymphadenopathy, and immunoblastic sarcoma are related disorders reflecting a clonal nature of immunoblastic lymphoma cells and the plasmacytic cells.