A patient with M-proteinemia (IgM, kappa type), lymphocytosis, anemia,
and massive splenomegaly, was diagnosed as having Waldenstrom's macro
globulinemia (WM). Since this case was refractory to chemotherapy, spl
enic irradiation was performed, which effectively reduced the serum Ig
M level, spleen size, and lymphocyte counts; however, its effect was t
ransient, Splenectomy was then carried out. The spleen contained abund
ant IgM-producing lymphocytes, and after splenectomy, the serum IgM va
lues 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 r
emission for six years after splenectomy. The removal of an IgM-produc
ing/secreting site and release from hypersplenism may be the major mec
hanisms involved in achieving the durable remission after splenectomy.
In individual cases of WM with massive splenomegaly, we recommend spl
enectomy as part of the management of this disorder.