Abstract
Two cases of macroglobulinemia, 65-year-old female and 59-year-old male, associated with obvious destructive bone lesions caused by neoplastic proliferation of plasma cells were reported.
Classical criteria for primary macroglobulinemia of Waldenström were scored by 1) presence of monoclonal gammopathy of IgM type, 2) proliferation of lymphoid or plasmocytoid lymphocytes, 3) lack of punched-out bone lesion, 4) high incidence of hepato-splenomegaly and of lymphadenopathy, 5) hyperviscosity syndrome associated with bleeding tendency and 6) relative benign course of the disease. Especially the lack of punched out bone lesion and proliferation of plasmocytoid lymphocytes, instead of plasma cells in multiple myeloma, are stressed repeatedly to differentiate the primary macroglobulinemia from multiple myeloma. Both cases reported here showed obvious punahed-out bone lesions, caused by proliferation of plasma cells, which were confirmed by X-ray bone survey and by autopsy.
Recently, three cases of macroglobulinemia associated with apparent bone lesion were reported among 80 reported cases of primary macroglobulinemia in Japan. characteristic findings of those cases were the lack of hepato-splenomegaly and lymphadenopathy, and marked neoplastic proliferation of plasma cells in bone marrow. The neoplastic proliferation of plasma cells associated with punched-out bone lesions are the charcteristic fiindings for multiple myeloma rather than that of primary macroglobulinemia.
The presence of IgM-secreting plasma cell clone has been confirmed by several investigators. Thus, those cases with monoclonal IgM associated with punched-out bone lesions caused by neoplastic proliferation of plasma cells should be defined as “Multiple myeloma of IgM type”.