首页> 外文会议>International Symposium on Amyloidosis >AMYLOID TUMORS OF BREAST, LUNG AND RIB IN NONSECRETORY MULTIPLE MYELOMA
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AMYLOID TUMORS OF BREAST, LUNG AND RIB IN NONSECRETORY MULTIPLE MYELOMA

机译:乳腺癌,肺和肋骨中的淀粉样肿瘤在非分泌多发性骨髓瘤中

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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.
机译:Nonsecretory多发性骨髓瘤(NSMM)代表多种骨髓瘤的变体,其特征在于血清和尿液中的unde-textable单克隆免疫球蛋白,并且占所有MM病例的一〜5%。由于NSMM和淀粉样源之间的罕见关联,以下案件是令人兴趣的。由于痛苦呼吸的6个月历史,49岁的妇女在1996年5月入院。在体检时,右侧肋骨上存在局部压痛。假定骨折。 X射线,计算机断层扫描和磁体共振成像显示右侧第七肋骨上的单一裂解病变。她过去的历史包括双方腕管综合征,具有以下外科手术。由于疑似骨溶解转移,肋骨肿瘤已被切除。组织病理学检查揭示了具有广泛淀粉样蛋白症的血浆细胞瘤细胞瘤。骨髓活检显示出80%成熟的小浆细胞的大规模渗透。实验室值是血红蛋白6,9mmol / L,白细胞5400 / UL和血小板306000 / UI。电解质,肌酸素,肝脏参数和β-2-微凝胶蛋白是正常的。定量免疫球蛋白研究检测到低血清IgG值4,75g / L(正常8-18g / L),IgA 0,14g / L)和IgM 0.18g / L的血清Igg值(0.9-4.4g / L)和IgM 0.18g / L. (0,45-2.5 g / l)。血清和尿液蛋白电泳对单克隆术语是阴性的。免疫电泳也未能显示任何单克隆免疫球蛋白。浓缩尿液的免疫混膜检测到κ型的轻链。制作了非分泌多发性骨髓瘤的诊断。启动了用梅酚和泼尼松治疗。在6个月的治疗后进行的骨髓抽吸显示仅显示25%的血浆细胞。这种疗法之后是干扰素A,含替代蛋白(Zavedos〜R)和地塞米松。用同种异体骨髓移植完成治疗。诊断4年后,患者再次入院,因为她开发了咳嗽和呼吸急促。计算机断层扫描在两个上部肺裂片中显示出纤维化。乳房X线照相表明可能的乳腺病变。肺和乳房活组织检查显示多个淀粉样肿瘤。刚果红染色显示各向异性和绿色偏振颜色。

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