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伴有类风湿样淀粉样关节病及血清中μ重链片段的多发性骨髓瘤(轻链病)

Multiple myeloma (light chain disease) with rheumatoid-like amyloid arthropathy and mu-heavy chain fragment in the serum.

作者信息

Pruzanski W, Hasselback R, Katz A, Parr D M

出版信息

Am J Med. 1978 Aug;65(2):334-41. doi: 10.1016/0002-9343(78)90828-8.

Abstract

A 52 year old man presented with multiple myeloma and widespread amyloidosis involving joints, bursae- carpal tunnels, lymph nodes and subcutaneous tissue. Osteolytic bone lesions and pathologic fracture of the neck of the left femur were found. Bone marrow was infiltrated with both plasmacytes and lymphocytes. The majority of plasma cells contained large cytoplasmic vacuoles. Free kappa II type light chains and mu-heavy chain fragment were detected in the serum, and kappa II type Bence Jones protein was found in the urine. The molecular weight of the mu-heavy chain fragment was found to be approximately 50,000 daltons. An immunofluorescence study, using the double labelling technic, showed that the majority of plasmacytes in bone marrow contained both mu and kappa antigenic determinants in the cytoplasm. A review of the 12 previously described patients with mu-heavy chain fragment in the serum showed a variable clinical picture, thus their natural history and therapeutic response are difficult to evaluate at the present time. It seems that mu-heavy chain fragment can be found in a variety of clinical conditions and that mu-heavy chain disease cannot yet be accepted as a separate clinical entity.

摘要

一名52岁男性患者,患有多发性骨髓瘤及累及关节、滑囊、腕管、淋巴结和皮下组织的广泛性淀粉样变性。发现溶骨性骨病变及左股骨颈病理性骨折。骨髓中既有浆细胞浸润,也有淋巴细胞浸润。大多数浆细胞含有大的胞质空泡。血清中检测到游离κII型轻链和μ重链片段,尿液中发现κII型本-周蛋白。μ重链片段的分子量约为50,000道尔顿。采用双重标记技术的免疫荧光研究显示,骨髓中的大多数浆细胞在胞质中同时含有μ和κ抗原决定簇。对先前描述的12例血清中有μ重链片段的患者进行回顾,发现临床表现各异,因此目前难以评估其自然病程和治疗反应。似乎μ重链片段可在多种临床情况下发现,且μ重链病目前尚不能被视为一个独立的临床实体。

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