単クローン性高γグロブリン血症(マクログロブリン血症)をしめしたIBL-like T cell Lymphomaの一例

抄録

A case of IBL like T cell lymphoma with monoclonal increasing IgM and κ-light chain in the serum was reported.
58 yrs female, who had been suffered from heart failure, admitted for the lymphadenopathy and general fatigue with fever. Leucopenia with small amount of atypical lymphocytes and hyperviscousity of blood were detectable. Serum analysis showed the monoclonal elevation of IgM-κ(M-protein) remarkably and slight dinturbance of liver function. Urinary Bence-Jones protein was detected. Lymph node biopsy revealed the disappearance of normal structure and the proliferation of atypical lymphoids with pale cells which characterize IBL like T cell lymphoma. Immunocytochemistry disclosed the pale cells bearing T cell markers (MT1, CD5, CD8 & CD4) and moderate distribution of IgM positive lymphoids and plasma cells. On November 1987, tonsil was swelling and was biopsied. Specimen showed the infiltration of atypical lymphoids and pale cells. Bone marrow biopsy showed moderate proliferation of lymph-plasmacytoid cells and formation of lymphoid germinal center. Clinical data and symptoms suggested the macroglobulinemia.
Additional lymph node biopsy was carried out on December 1987, and specimen also revealed IBL like T cell lymphoma. Monoclonal increasing of IgM-has been still remained. The patient is fortunately alive and well irrespective of no-recieving of therapy for lymphoma. IBL like T cell lymphoma is characterized with polyclonal hypergammaglobulinemia. The present case representing as IBL like T cell lymphoma histologically, however, showed monoclonal gammopathy which was macroglobulinemia clinically. It is difficult to define the pathogenesis whether IBL like T cell lymphoma induce the macroglobulinemia or the two kinds of the diseases occur simultenously and independently.