Nonsecretory multiple myeloma (NSMM) represents a variant of multiple myeloma characterized by unde-tectable monoclonal immunoglobulins in serum and urine, and accounts for one to five percent of all cases of MM. The following case is of interest because of the rare association between NSMM and amyloidosis.A 49-year-oid woman was hospitalized in May 1996 because of a 6-month history of painful breathing. On physical examination, there was a localized tenderness on the right seventh rib. A fracture was presumed. X-ray, computed tomography and magnet resonance imaging showed a single lytic lesion on the right seventh rib. Her past history included a carpal tunnel syndrome on both sides with following surgical procedure. The rib tumor has been resected because of a suspected osteolytic metastasis. The histopathologic examination revealed a plasmocytic plasmocytoma with extensive amyloidosis. Bone marrow biopsy showed a massive infiltration of 80% mature small plasma cells. Laboratory values were hemoglobin 6,9 mmol/l, leucocytes 5400/ul and thrombocytes 306000/ui. Electrolyte, creatinin, liver parameters and beta_2-microglobulin were normal. Quantitative immunoglobulin studies detected hypogammaglobulinemia with a serum IgG value of 4,75 g/l (normal 8-18 g/l), IgA 0,14 g/l (0.9-4.4 g/l) and IgM 0,18 g/l (0,45-2,5 g/l). Serum and urine protein electrophoresis were both negative for monoclonal gammopathy. Also immunoelectrophoresis failed to show any monoclonal immunoglobulins. Immunofixation of the concentrated urine however detected light chains of kappa-type. A diagnosis of nonsecretory multiple myeloma was made. Treatment with melphalan and prednisone was initiated. A bone marrow aspiration performed after 6 month of therapy showed only 25% plasma cells. This therapy was followed by interferon a, idarubicin (Zavedos~R) and dexamethasone. Therapy was completed with an allogenic bone marrow transplantation. Four years after diagnosis the patient was admitted to the hospital again because she developed cough and shortness of breath. Computed tomography showed a fibrosis in both upper pulmonary lobes. Mammography indicated a possible mastopathy. A pulmonary and breast biopsy revealed multiple amyloid tumors. Congo red staining showed an anisotropy and green polarization color.
展开▼