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B细胞淋巴增殖性疾病中的低分子量免疫球蛋白M

Low molecular weight IgM in B cell lymphoproliferative disorders.

作者信息

Roberts-Thomson P J, Jones D N, Koh L Y, Neoh S H, Thomas M, Bradley J

出版信息

Aust N Z J Med. 1984 Apr;14(2):121-5. doi: 10.1111/j.1445-5994.1984.tb04273.x.

Abstract

Circulating low molecular weight (LMW) IgM was demonstrated in five of 38 patients with B cell lymphoproliferative disorders. These five patients all had malignant disease and could be subdivided into two groups. In the first group were three patients, each with an associated serum IgM paraprotein; two had Waldenström's macroglobulinemia, and one lymphocytic lymphoma. The two patients of the second group did not have IgM paraproteins; one had lymphocytic lymphoma and one chronic lymphocytic leukemia. Both these patients also had acquired C1 esterase inhibitor deficiency, a previously recognised association with circulating LMW IgM. None of the 16 patients with benign IgM macroglobulinemia had circulating LMW IgM. In those positive sera with LMW IgM this moiety contributed between 10.5% and 37.5% of the total IgM. There was no apparent association between LMW IgM and total IgM levels, kappa/lambda typing or the presence of Bence Jones proteinuria, but rheumatoid factor, immune complexes and cryoglobulins occurred in many of the sera which contained LMW IgM. Pokeweed mitogen stimulated peripheral blood mononuclear cells from two patients with circulating LMW IgM secreted considerable quantities of this moiety in vitro but this did not occur in two patients with benign IgM macroglobulinemia. We conclude that LMW IgM is found in the malignant but not the benign forms of B cell lymphoproliferative disorders and is frequently associated with other serological abnormalities. The basic abnormality causing defective IgM polymerisation in these disorders is obscure.

摘要

在38例B细胞淋巴增殖性疾病患者中,有5例检测到循环低分子量(LMW)IgM。这5例患者均患有恶性疾病,可分为两组。第一组有3例患者,每例均伴有血清IgM副蛋白;2例患有华氏巨球蛋白血症,1例患有淋巴细胞淋巴瘤。第二组的2例患者没有IgM副蛋白;1例患有淋巴细胞淋巴瘤,1例患有慢性淋巴细胞白血病。这2例患者还均患有获得性C1酯酶抑制剂缺乏症,这是一种先前已确认的与循环LMW IgM相关的疾病。16例良性IgM巨球蛋白血症患者均未检测到循环LMW IgM。在那些含有LMW IgM的阳性血清中,该部分占总IgM的10.5%至37.5%。LMW IgM与总IgM水平、κ/λ分型或本周蛋白尿的存在之间没有明显关联,但类风湿因子、免疫复合物和冷球蛋白在许多含有LMW IgM的血清中出现。美洲商陆有丝分裂原刺激来自2例循环LMW IgM患者的外周血单个核细胞在体外分泌大量该部分,但2例良性IgM巨球蛋白血症患者未出现这种情况。我们得出结论,LMW IgM存在于B细胞淋巴增殖性疾病的恶性形式而非良性形式中,并且经常与其他血清学异常相关。在这些疾病中导致IgM聚合缺陷的基本异常尚不清楚。

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