A patient with M-proteinemia (
IgM, kappa type),
lymphocytosis,
anemia, and massive
splenomegaly, was diagnosed as having Waldenström's
macroglobulinemia (WM). Since this case was refractory to
chemotherapy, splenic irradiation was performed, which effectively reduced the serum
IgM level, spleen size, and lymphocyte counts; however, its effect was transient.
Splenectomy was then carried out. The spleen contained abundant
IgM-producing lymphocytes, and after
splenectomy, the serum
IgM values decreased and the peripheral blood counts returned to near normal. The transient increases of serum
IgM occurred during two infectious episodes postoperatively. The patient has now been in a satisfactory remission for six years after
splenectomy. The removal of an
IgM-producing/secreting site and release from
hypersplenism may be the major mechanisms involved in achieving the durable remission after
splenectomy. In individual cases of WM with massive
splenomegaly, we recommend
splenectomy as part of the management of this disorder.