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短期血液透析后非典型β2-微球蛋白淀粉样变性

Atypical beta 2-microglobulin amyloidosis following short-term hemodialysis.

作者信息

Albers S E, Fenske N A, Glass L F, Brozena S J, Szakacs J E

机构信息

Division of Dermatology & Cutaneous Surgery, University of South Florida College of Medicine, Tampa 33612.

出版信息

Am J Dermatopathol. 1994 Apr;16(2):179-84. doi: 10.1097/00000372-199404000-00014.

Abstract

A 47-year-old white female renal transplant recipient presented complaining of "wrinkles" that predominantly involved the palmar aspect of her fingers. Light-microscopic examination of biopsy material obtained from affected areas revealed deposits of amorphous, hyaline material in the reticular dermis that contrasted sharply with collagen and elastic fibers. Examination by transmission electron microscopy disclosed a mass of fibrils recognizable as amyloid, despite negative histochemical staining with both Congo red and crystal violet. Immunoperoxidase staining for beta 2-microglobulin was positive. Along with an elevated serum beta 2-microglobulin level, this finding supported the diagnosis of hemodialysis-induced amyloidosis (HIA). Unusual features of our case include lesion location, short duration of antecedent hemodialysis therapy, and negative staining for amyloid using conventional staining techniques.

摘要

一名47岁的白人女性肾移植受者前来就诊,主诉主要累及手指掌面的“皱纹”。对受累部位获取的活检材料进行光镜检查,发现网状真皮中有无定形、透明样物质沉积,与胶原纤维和弹性纤维形成鲜明对比。透射电子显微镜检查显示有大量可识别为淀粉样蛋白的原纤维,尽管刚果红和结晶紫组织化学染色均为阴性。β2微球蛋白免疫过氧化物酶染色呈阳性。结合血清β2微球蛋白水平升高,这一发现支持血液透析相关性淀粉样变(HIA)的诊断。我们病例的不寻常特征包括病变部位、先前血液透析治疗时间短以及使用传统染色技术对淀粉样蛋白染色阴性。

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