Nobuo Takemori

Successful treatment of immunoblastic lymphadenopathy-like T-cell lymphoma with cyclosporin A.

Immunoblastic lymphadenopathy (IBL)-like T-cell lymphoma is considered to belong to peripheral T-cell lymphoma. Its prognosis is grave and effective treatments have not been established. Recently, we gave oral cyclosporin A (CsA) to a patient with IBL-like T-cell lymphoma, and succeeded in achieving dramatic remission. In this case, serum levels of interleukin-12 (IL-12) and tumor necrosis factor-alpha (TNF alpha) were elevated and decreased or returned to normal after achieving remission. Since CsA is a potent suppressor of the immune system and most notably T-cells, the immunosuppression of T-cell function might have played an important role in achieving remission in this case, although the precise mechanism still remains to be elucidated. The present case indicates that administration of CsA may be a very effective and safe selection of therapy for IBL-like T-cell lymphoma, as well as analogous disorders such as IBL and angioimmunoblastic lymphadenopathy with dysproteinemia (AILD), thereby will contribute to improving the prognosis of patients with these diseases.

DURABLE REMISSION AFTER SPLENECTOMY FOR WALDENSTROM'S MACROGLOBULINEMIA WITH MASSIVE SPLENOMEGALY IN LEUKEMIC PHASE

A patient with M-proteinemia (IgM, kappa type), lymphocytosis, anemia, and massive splenomegaly, was diagnosed as having Waldenströms 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.

PLURAL IMMUNOGLOBULIN SYNTHESIS IN A SINGLE CELL : AN ULTRASTRUCTURAL STUDY OF TWO CASES WITH THREE M-PROTEINS

Electrophoresis revealed two cases of malignant lymphoma that each contained three M-proteins (IgM lambda.lgG kappa.lgG lambda and IgM lambda.IgM kappa.lgG kappa) in the sera. To determine cellular origin of each M-protein, atypical lymphoid and plasmacytoid cells of both cases were examined by electron microscopy. Atypical lymphoid and plasmacytoid cells possessed rough endoplasmic reticula (RERs) in varying degrees, as seen by conventional electron microscopy, and showed double-stainability for plural antibodies against immunoglobulins following double stainings of immunoelectron microscopy using immunogold staining. Rabbit antibodies against human IgM, lgG, free kappa-light chain and free lambda-light chain were used for the immunoelectron microscopic staining. By the double staining method, plural immunoglobulins, IgM/IgG, IgM/free kappa, IgM/free lambda, IgG/free kappa, IgG/free lambda and free kappa/free lambda, were simultaneously detected in varying degrees in the Golgi area, RERs, and dense bodies of lymphoid and plasmacytoid cells. In conclusion, this study directly exhibited, through electron microscopy, that plural immunoglobulins were synthesized at the same time in a single cell, and that the process of immunoglobulin synthesis in the lymphoid and plasmacytoid cells was different from that in a normal B-cell.