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慢性淋巴细胞白血病和恶性组织细胞瘤患者中的μ链病。I. 临床方面。

mu-chain disease in a case of chronic lymphocytic leukaemia and malignant histiocytoma. I. Clinical aspects.

作者信息

Jonsson V, Videbek A, Axelsen N H, Harboe M

出版信息

Scand J Haematol. 1976 Mar;16(3):209-17. doi: 10.1111/j.1600-0609.1976.tb01140.x.

DOI:10.1111/j.1600-0609.1976.tb01140.x
PMID:179130
Abstract

A 27-year-old woman with proteinuria and normal serum creatinine for 10 years developed chronic lymphocytic leukaemia (CLL) and after further 2 years during treatment with prednisone and chlorambucil a malignant histiocytoma appeared. Free mu-chains were demonstrated in serum, but it is possible that mu-chain disease (mu-CD) and Bence-Jones proteinuria had persisted unrecognized for 12 years, since vacuolated plasma cells were found when the primary bone marrow smear was re-evaluated and no glomerular disorders were found. The clinical findings from the now 11 known cases of mu-CD are reveiwed and the immunochemical findings are reported ain part II (Axelsen et al 1976). The patient's mother died of acute leukaemia at the age of 24. The mother's sister has mammary carcinoma and several members of the mother's line apparently have increased tendency to infections. Of 27 family members, none exhibited immunoglobulin defects, but 10 members of the mother's line had B- as well as T-lymphocytosis and 5 members had leukocytosis.

摘要

一名27岁女性,蛋白尿10年,血清肌酐正常,后发展为慢性淋巴细胞白血病(CLL),在使用泼尼松和苯丁酸氮芥治疗2年后出现恶性组织细胞瘤。血清中检测到游离μ链,但有可能μ链病(mu-CD)和本-周蛋白尿已持续未被识别达12年,因为重新评估初次骨髓涂片时发现有空泡化浆细胞,且未发现肾小球疾病。本文回顾了目前已知的11例mu-CD的临床病例,并在第二部分(Axelsen等人,1976年)报告了免疫化学结果。患者的母亲在24岁时死于急性白血病。母亲的妹妹患有乳腺癌,母亲家族的几名成员显然有感染倾向增加的情况。在27名家庭成员中,没有人表现出免疫球蛋白缺陷,但母亲家族的10名成员同时存在B淋巴细胞增多和T淋巴细胞增多,5名成员有白细胞增多。

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Successful treatment of mu-heavy chain disease with fludarabine monophosphate: a case report.单磷酸氟达拉滨成功治疗μ重链病:一例报告
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A case of gamma 3 heavy chain disease with vacuolated plasma cells: a clinical, immunological, and ultrastructural study.1例伴有空泡化浆细胞的γ3重链病:一项临床、免疫学及超微结构研究
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