Durable Remission after Splenectomy for Waldenström's Macroglobulinemia with Massive Splenomegaly in Leukemic Phase

Abstract

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.

Multicenter, randomized comparative trial of fludarabine and the combination of cyclophosphamide-doxorubicin-prednisone in 92 patients with Waldenström macroglobulinemia in first relapse or with primary refractory disease

“…[27][28][29] Previous trials of second-line treatments involved doxorubicin, high-dose corticosteroids, ␥ interferon, and splenectomy, but these studies were limited and the results not particularly noteworthy. [16][17][18]30,31 We chose CAP as the comparative regimen in this study because of its efficacy in low-grade lymphoid malignant diseases such as chronic lymphocytic leukemia and low-grade lymphoma. 32,33 Furthermore, Dimopoulos and Alexanian 3 observed similar response rates in 20 patients who received first-line treatment with cyclophosphamide, hydroxydaunomycin, vincristine, and prednisone and in a historical series of patients treated with a combination of chlorambucil and prednisone, but there was significant difference in median survival rates.…”

Advanced Waldenström’s Macroglobulinemia: A Case of Possible Cure after Systemic Chemotherapy, Splenic Radiation and Splenectomy

“…The function of the spleen and the significance of splenectomy in WM were described by Takemori et al [17]. The spleen is known to have diverse and sophisticated functions including immune function, a filter function, a blood volume-regulating function, a reservoir function [17].…”

“…The function of the spleen and the significance of splenectomy in WM were described by Takemori et al [17]. The spleen is known to have diverse and sophisticated functions including immune function, a filter function, a blood volume-regulating function, a reservoir function [17]. Splenectomy performed in humans for trauma or illness causes a decrease of serum IgM concentration [18, 19, 20]and an impaired IgM response to pneumococcal vaccine [21].…”

Splenectomy in plasma cell dyscrasias: A review of the clinical practice

“…Splenectomy led to amelioration of the abdominal symptoms and cytopenias in all cases. Takemori et al [19] reported a case of IgM k type WM with massive splenomegaly, in which splenectomy was performed as a last resort in the management of the disease that was resistant to chemotherapy, and showed only a transient response to splenic irradiation. IgM values and the complete blood count returned to near normal limits within 3 months of the removal of the spleen.…”

“…The decrease in the production of these blocking factors, along with release from hypersplenism, and removal of a favored site for IgM production/secretion, are all considered to contribute in achieving satisfactory remission in splenectomized patients [10,19,20].…”