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骨髓瘤:一例罕见表现的病例报告。

Myelomatosis: a case report of an unusual presentation.

作者信息

Shokunbi W A, Raji A A, Fakunle E E

机构信息

Department of Haematology, University College Hospital, Ibadan.

出版信息

West Afr J Med. 2006 Oct-Dec;25(4):312-5.

Abstract

BACKGROUND

Multiple myeloma (MM) is a clonalexpansion of plasma cell characterized by skeletal dissemination of malignant plasma cells resulting in the production of homogenous monoclonal immunoglobulin termed the monoclonal (M) protein. MM is the most prevalent cancer after non-Hodgkin's lymphoma and is responsible for 2% of all cancer deaths. Several unusual presentation of MM have been described in the literature. Many serial reports have documented progression of Plasmacytoma to MM, or relapsed into MM after radiation therapy.

STUDY DESIGN

This is a case report of a 36 yr old man with a huge fungating right clavicular mass, with a histological of plasmacytoma (anaplastic variant).

RESULT

The patient's tumour was resistant to radiotherapy, terminating as multiple myeloma, with rapid progression to death within 19 months post diagnosis.

CONCLUSION

This report helps to validate the rapid progression of the anaplastic plasmacytoma variant to disseminated myeloma, and indicates the poor response to radiation therapy. Our patient showed the important poor prognostic features in some plasmacytomas that are characterized by rapid progression to MM, namely tumour size > 5 cm, young age and spinal cord compression.

摘要

背景

多发性骨髓瘤(MM)是浆细胞的克隆性增殖,其特征为恶性浆细胞在骨骼中播散,导致产生称为单克隆(M)蛋白的同质单克隆免疫球蛋白。MM是继非霍奇金淋巴瘤之后最常见的癌症,占所有癌症死亡人数的2%。文献中描述了MM的几种不寻常表现。许多系列报告记录了浆细胞瘤进展为MM,或在放射治疗后复发为MM。

研究设计

这是一例36岁男性的病例报告,其右锁骨有一个巨大的溃疡性肿块,组织学检查为浆细胞瘤(间变性变体)。

结果

患者的肿瘤对放疗耐药,最终发展为多发性骨髓瘤,在诊断后19个月内迅速进展至死亡。

结论

本报告有助于证实间变性浆细胞瘤变体迅速进展为播散性骨髓瘤,并表明对放射治疗反应不佳。我们的患者在一些浆细胞瘤中表现出重要的不良预后特征,其特征为迅速进展为MM,即肿瘤大小>5 cm、年轻和脊髓受压。

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