Waldenstrom's Macroglobulinemia : report of 8 cases

Abstract

Background: Waldenstrom's Macroglobulinemia (WM) is a lymphoproliferative disorder characterized by neoplastic B-lymphocytes infiltrating the bone marrow and other lymphoid organs with the production of monoclonal IgM protein. Patients and Methods: In this report, we review the cases of WM seen at IRCH, AIIMS, New Delhi, in the last 5 years. Criteria for diagnosis included serum monoclonal IgM protein along with 20 percent bone marrow lymphocytes. Eight cases were included in the study with a median age of 59.5 years at diagnosis and male/female ratio 7:1. Two patients were asymptomatic at diagnosis. The common clinical features at presentation were fatigue (100 percent), malaise (100 percent), bleeding tendencies (66.6 percent), symptoms suggestive of hyperviscosity (33.3 percent). Anemia was seen in 75 percent cases, hepatomegaly in 37.5 percent cases, splenomegaly in 25 percent cases, lymphadenopathy in 12.5 percent cases. None had peripheral neuropathy. Laboratory investigations revealed a mean Hb8.1g/dl, mean TLC 6.2' 10/1, mean platelet count 1.09 ' 10/1 and mean ESR 100.6 mm/hr. 75 percent cases had diffuse bone marrow involvement by lympho/lymphoplasmacytoid cells. The mean serum M spike concentration was 3.18g/dl and all the patients had IgM kappa subtype. As initial therapy, 5 patients received Chlorambucil/Prednisolone, 1 patient received VAD chemotherapy and the other 2 asymptomatic patients were observed without any therapy. None had complete response. 2 had partial response while 3 had minimal response. One of the asymptomatic case progressed after a period of 18 months of observation. 7 out of 8 cases were alive at the time last follow up. The overall median survival was 19.5 months and progression free survival was 16.3 months. Conclusion: Though a rare disorder, high clinical suspicion is required to diagnose WM. Therapy with Chlorambucil/Prednisolone provides adequate disease control.