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儿童多发性骨髓瘤。

Multiple myeloma in a child.

作者信息

Bernstein S C, Perez-Atayde A R, Weinstein H J

出版信息

Cancer. 1985 Oct 15;56(8):2143-7. doi: 10.1002/1097-0142(19851015)56:8<2143::aid-cncr2820560844>3.0.co;2-8.

Abstract

A 12-year-old girl with the diagnosis of multiple myeloma is described. She presented with a nasopharyngeal mass which was histologically found to be a plasmacytoma. Serum immunoelectrophoresis revealed an IgA-kappa M-protein (4.9 g/dl). There were approximately 20% atypical plasma cells in a bone marrow biopsy specimen. The diagnosis was further supported by immunohistochemical demonstration of cytoplasmic monoclonal IgA-kappa in the tumor cells of both the nasopharyngeal and bone marrow biopsies. The patient was treated with chemotherapy for 1 year, at which time she became refractory to treatment, based on serum IgA levels. Five months after cessation of therapy, she continues to exhibit a significant objective response, remaining clinically well with a stable, elevated serum IgA level.

摘要

本文描述了一名诊断为多发性骨髓瘤的12岁女孩。她表现为鼻咽部肿物,组织学检查发现为浆细胞瘤。血清免疫电泳显示IgA-κM蛋白(4.9g/dl)。骨髓活检标本中约有20%的非典型浆细胞。鼻咽部和骨髓活检肿瘤细胞中细胞质单克隆IgA-κ的免疫组化证实进一步支持了诊断。该患者接受了1年的化疗,基于血清IgA水平,此时她对治疗产生了耐药。停止治疗5个月后,她仍表现出显著的客观反应,血清IgA水平持续稳定升高,临床状况良好。

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