Letter to the Editor: Splenectomy for a Case with Waldenstrom Macroglobulinemia With Giant Splenomegaly
Splenomegaly is a common finding in Waldenstrom macroglobulinemia as a part of the multisystemic disease. However, a case showing giant splenomegaly as a sole initial finding has been so far rarely reported. Here, we describe a patient with WM having giant splenomegaly who achieved a striking response to splenectomy.
The patient was a 53-year-old man who had been well until February 1985, when he first experienced easy fatiguability and malaise. Mild splenomegaly and liver dysfunction were found, but he received no further evaluation. In August 1987, he was admitted to our hospital because of a sense of abdominal fullness and Raynaud symptoms. Physical examination revealed anemia, a palpable spleen to 17 cm below the left costal margin and no appreciable lymphadenopathy. The complete blood count (CBC) revealed hemoglobin 9.3 g/dl, platelets 72,OOO/pl, and leukocytes 3,2001pl with 70% mature lymphocytes. A bone marrow aspirate was normocellular with increased numbers (32%) of lymphocytes and lymphoplasmacytoid cells. Total serum protein was 9.1 g/dl with 41.5% gammaglobulin. Quantitative immunoglobulins were as follows: IgG 2,544 mg/dl, IgA 226 mg/dl, and IgM 2,220 mg/dl. Immunoelectrophoresis showed a monoclonal IgM paraprotein (K type) in the serum. The diagnosis of Waldenstrom macroglobulinemia was made and he was treated with combination chemotherapy consisting of melpharan, prednisolone, vincristine, and cyclophophamide. The chemotherapy initially led to symptomatic improvements, but in March 1989 severe pancytopenia and visual disturbances developed. The IgM protein had increased to 3,120 mg/dl. An exploratory laparotomy and splenectomy were performed. The spleen was markedly enlarged and weighed 2,820 g. Microscopic examination showed a great proliferation of lymphocytes, plasma cells, and transitional lymphoplasmacytoid cells in the white pulp with expansion into the red pulp. Postoperative recovery was uneventful, and pancytopenia disappeared in a short time. From 3 months after operation, the patient has received 8 mglday melpharan and 40 mg/day prednisolone for 5 days every 1.5 month and he remained free of symptoms for 20 months. The last laboratory findings were as follows: hemoglobin 14.9 g/dl, platelets 203,00O/pl, leukocytes 5,OOO/pl with normal differentials, and IgM 1,630 mg/dl.