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IgE 多发性骨髓瘤

IgE multiple myeloma.

作者信息

Macro M, André I, Comby E, Chèze S, Chapon F, Ballet J J, Reman O, Leporrier M, Troussard X

机构信息

Department of Clinical Haematology, Centre Hospitalier Universitaire, Caen, France.

出版信息

Leuk Lymphoma. 1999 Feb;32(5-6):597-603. doi: 10.3109/10428199909058419.

Abstract

IgE multiple myeloma is a rare disease characterized by a high frequency of Bence-Jones proteinuria and plasma cell leukaemia when compared to other isotypes of monoclonal proteins. Only 35 cases have been reported. We describe a 70-year-old woman with a stage III IgE kappa multiple myeloma presenting with a sacral plasmacytoma. Immunological and biochemical studies showed IgE kappa producing tumoral plasma cells. Serum total IgE was high without clinical symptoms suggesting an hyperIgE syndrome or mast cell activation. The patient underwent surgical removal of the sacral tumor and monthly melphalan-prednisone treatment together with intravenous pamidronate infusions. Magnetic Resonance Imaging (MRI) of the dorsolumbar spine revealed an epidural process leading to T6-T9 radiotherapy. Bone densitometry showed a decreased bone mineral content supporting the management of myeloma-related osteoporosis with bisphosphonate infusions. A good partial response with plateau-phase and increase of bone mineral content was achieved after 1 year of treatment and still persists after a 28 months follow-up.

摘要

与其他单克隆蛋白同种型相比,IgE型多发性骨髓瘤是一种罕见疾病,其特征是本-周蛋白尿和浆细胞白血病的发生率较高。仅报告了35例病例。我们描述了一名70岁女性,患有III期IgE κ型多发性骨髓瘤,伴有骶骨浆细胞瘤。免疫和生化研究显示产生IgE κ的肿瘤性浆细胞。血清总IgE升高,但无提示高IgE综合征或肥大细胞活化的临床症状。患者接受了骶骨肿瘤的手术切除,并每月接受美法仑-泼尼松治疗以及静脉注射帕米膦酸。胸腰椎磁共振成像(MRI)显示硬膜外病变,导致T6-T9放疗。骨密度测定显示骨矿物质含量降低,支持用双膦酸盐输注治疗骨髓瘤相关骨质疏松症。治疗1年后取得了良好的部分缓解,进入平台期,骨矿物质含量增加,在28个月的随访后仍持续存在。

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