A case of primary macroglobulinemia with apparent punched out bone lesions in skull and femur is reported.
The patient, 72 year-old female, was admitted to the Meijo Hospital because of severe lumbago and anemia. She developed bleeding manifestations such as melena, genital bleeding and retinal hemmorhage possibly due to thrombocytopenia. X-ray examination of bones disclosed definite punched out lesions in skull and femur and generalized osteoporosis. M-component found by electrophoresis on cellulose acetate membrane was identified as macroglobulin by immunoelectrophoresis. Levels of the macroglobulin with sedimentation constant of 16.7 S ranged from 900 to 1,300 mg/dl during the course of her disease. Relative viscosity of serum was within normal range.
Plasma cells constituted more than 80% of the total bone marrow cells and plasmacytoid lymphocytes were occasionally found especially at terminal stage. Tumor cells possessed an ovoid or rounded and peripherally displaced nucleus, exhibited abundant cytoplasm rich in endoplasmic reticulum, and had a prominent Golgi area. She died 15 months after the onset of the disease, and the infiltration of plasma cells to bones including sternum, thoracic and lumbar vertebrae and ileum as well as kidney, liver, pancreas, spleen and adrenal cortex was shown by autopsy.
Based on these findings, myelomatous macroglobulinemia was proposed as a rare form of primary macroglobulinemia.
