A case of typical diffuse plasmocytoma with IgM paraproteinemia is described. The patient presented with systemic and monomorphic proliferation of atypical and immature plasma cells, osteolytic lesions, retinal venous thrombosis and hemorrhagic diathesis. The therapy with steroids and alkeran produced clinical and hematological remission for 11/2 year with a decrease of macroglobulins in the serum. Cell and paraprotein type remained unchanged during the course of the disease. This case, and similar ones already reported in the literature, suggest that neoplastic plasma cell proliferation is accompanied by synthesis of paraproteins including the IgM type. However, this is not characteristic for Waldenström’s macroglobulinemia. The author concludes that differential diagnosis in these cases can only be made by cytologic examination.

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