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[多发性骨髓瘤病程中的淀粉样变性]

[Amyloidosis in the course of multiple myeloma].

作者信息

Mazur Grzegorz, Usnarska-Zubkiewicz Lidia, Wróbel Tomasz, Biedroń Monika, Nowicka Jadwiga, Ganczarski Grzegorz, Kuliczkowski Kazimierz

机构信息

Katedra i Klinika Hematologii, Nowotworów Krwi i Transplantacji Szpiku AM we Wrocławiu.

出版信息

Pol Arch Med Wewn. 2005 Apr;113(4):341-5.

Abstract

The amyloidoses are group of heterogeneous disorders, in which synthesized and secreted proteins, as a soluble molecules, are formed into insoluble, fibrillar tissue deposits, leading to organ dysfunction. Classification now is based on the chemical nature of the fibrillar component of the deposits. One of these is light-chain amyloidosis (AL). The aim of the study was to describe of multiple myeloma patients and amyloidosis. The study group consisted of 45 patients (16 men and 29 women). The diagnosis was made by fine-needle aspiration of subcutaneous fat and then staining the tissue with Congo red. We also analyse the concentration of the serum SAA. We analyse the most characteristic features of AL as hearth failure, proteinuria, renal failure, carpal tunnel syndrome, hepatosplenomegaly, macroglossia and orthostatic hypotension. Among the multiple myeloma patients we found 17 with AL amyloid and 35 persons with elevated concentration of the serum SAA. The most frequent symptoms were related with renal failure and heart failure.

摘要

淀粉样变性是一组异质性疾病,其中合成并分泌的蛋白质作为可溶性分子形成不溶性的纤维状组织沉积物,导致器官功能障碍。目前的分类基于沉积物纤维成分的化学性质。其中之一是轻链淀粉样变性(AL)。该研究的目的是描述多发性骨髓瘤患者和淀粉样变性。研究组由45名患者组成(16名男性和29名女性)。通过皮下脂肪细针穿刺并随后用刚果红对组织染色进行诊断。我们还分析了血清淀粉样蛋白A(SAA)的浓度。我们分析了AL的最典型特征,如心力衰竭、蛋白尿、肾衰竭、腕管综合征、肝脾肿大、巨舌和体位性低血压。在多发性骨髓瘤患者中,我们发现17例患有AL淀粉样变性,35例血清SAA浓度升高。最常见的症状与肾衰竭和心力衰竭有关。

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