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原发性浆细胞白血病伴广泛致密性骨硬化:联合化疗后完全缓解

Primary plasma cell leukemia with extensive dense osteosclerosis: complete remission following combination chemotherapy.

作者信息

Kuo M C, Shih L Y

机构信息

Department of Internal Medicine, Chang Gung Memorial Hospital, Taipei, Taiwan, R.O.C.

出版信息

Ann Hematol. 1995 Sep;71(3):147-51. doi: 10.1007/BF01702651.

Abstract

Diffuse osteosclerotic myeloma is very rare, and primary plasma cell leukemia with extensive osteosclerosis is even more rare. We describe a 71-year-old man who presented with severe anemia and dense widespread osteosclerosis similar to the X-ray finding of myelosclerosis. His peripheral blood showed 40% plasma cells. Bone marrow examination revealed heavy plasma cell infiltration with marked myelofibrosis and myelosclerosis. Protein electrophoresis and immunoelectrophoresis demonstrated an M-protein of IgG-lambda type. He was treated with cyclophosphamide, vincristine, and prednisolone for 10 months. A complete remission was obtained, with disappearance of M-protein and circulating plasma cells and normalization of complete blood counts, bone marrow picture, and biochemical parameters, as well as complete regression of myelofibrosis and osteosclerotic lesions. Unmaintained complete remission lasted for more than 1 year and he survived for more than 22 months. Our case indicated that one must include in the differential diagnosis of an osteosclerotic lesion the possibility of multiple myeloma, and that combination chemotherapy can induce a complete remission in this disease.

摘要

弥漫性骨硬化性骨髓瘤非常罕见,而伴有广泛骨硬化的原发性浆细胞白血病更为罕见。我们报告一例71岁男性,表现为严重贫血及广泛致密骨硬化,X线表现类似骨髓硬化。其外周血显示40%为浆细胞。骨髓检查发现大量浆细胞浸润,伴有明显的骨髓纤维化和骨硬化。蛋白电泳和免疫电泳显示为IgG-λ型M蛋白。患者接受环磷酰胺、长春新碱和泼尼松龙治疗10个月。获得完全缓解,M蛋白和循环浆细胞消失,全血细胞计数、骨髓象及生化指标恢复正常,骨髓纤维化和骨硬化病变完全消退。未维持的完全缓解持续超过1年,患者存活超过22个月。我们的病例提示,在骨硬化性病变的鉴别诊断中必须考虑多发性骨髓瘤的可能性,联合化疗可使该病获得完全缓解。

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