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干燥综合征、混合性冷球蛋白血症及华氏巨球蛋白血症(作者译)

[Sjögren's syndrome, mixed cryoglobulinemia and Waldenström's purpura (author's transl)].

作者信息

Celada A

出版信息

Med Clin (Barc). 1979 Sep 15;73(5):190-3.

PMID:491786
Abstract

A 66-year-old patient was hospitalized after the appearance of a deep ulcer in the posterior face of the right calf. She had purpuric spots on the lower limbs only, as well as parotiditis, especially on the left side, and was affected with dryness of the mouth but not of the eyes. The patient also presented, in contact with the cold, Raynaud's phenomenon. Physical examination revealed as pathologic signs: purpura of the lower limbs, a deep ulceration on the right calf, parotiditis, and a reduction in the pallaesthesia of the lower limbs. Biologically an increased sedimentation rate, elevation of the total proteins (particularly the gammaglobulins), positive antinuclear factor, reduction of the total complement and of the C3 fraction, circulating immunocomplexes, positive latex test, positive Sia test, and the presence of mixed cryoglobulin IgG-IgM type kappa, stood out. The patient was diagnosed as having Sjögren's syndrome, mixed cryoglobulinemia and Waldenström's purpura. Probably the two latter conditions characterized by the presence or circulating immunocomplexes are part of the same process. According to recently studies described in the literature the relationships between these three entities are discussed, as well as the possibility of developing lymphoproliferative syndromes under certain immune stimuli.

摘要

一名66岁患者因右小腿后侧出现深部溃疡而住院。她仅下肢有紫癜斑,还有腮腺炎,尤其是左侧,伴有口干但眼不干。患者还存在遇冷时的雷诺现象。体格检查显示有以下病理体征:下肢紫癜、右小腿深部溃疡、腮腺炎以及下肢触觉减退。实验室检查发现血沉加快、总蛋白(尤其是γ球蛋白)升高、抗核因子阳性、总补体及C3成分降低、循环免疫复合物、乳胶试验阳性、Sia试验阳性以及存在κ型IgG-IgM混合型冷球蛋白。该患者被诊断为干燥综合征、混合性冷球蛋白血症和瓦尔登斯特伦紫癜。后两种以循环免疫复合物存在为特征的病症可能是同一过程的一部分。根据文献中描述的近期研究,讨论了这三种病症之间的关系,以及在某些免疫刺激下发生淋巴增殖性综合征的可能性。

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