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华氏巨球蛋白血症伴AA型淀粉样变性

Waldenström's macroglobulinemia associated with AA amyloidosis.

作者信息

Gardyn J, Schwartz A, Gal R, Lewinski U, Kristt D, Cohen A M

机构信息

Hematology Unit, Rabin Medical Center, Petah-Tikva, Israel.

出版信息

Int J Hematol. 2001 Jul;74(1):76-8. doi: 10.1007/BF02982553.

Abstract

It is widely accepted that amyloidosis in Waldenström's macroglobulinemia (WM) is exclusively due to amyloid light-chain deposition. However, only a small number of previous reports have actually characterized the type of amyloid in WM. We now report the third patient with WM and amyloid A protein (AA) amyloidosis. This patient developed malabsorption, nephrotic syndrome, and orthostatic hypotension. AA was immunohistochemically demonstrated in the rectal biopsy. In conjunction with previous examples of AA amyloidosis, the present report raises the possibility that AA amyloidosis may also occur in WM patients.

摘要

人们普遍认为,华氏巨球蛋白血症(WM)中的淀粉样变性完全是由淀粉样轻链沉积引起的。然而,之前仅有少数报告实际对WM中的淀粉样蛋白类型进行了特征描述。我们现在报告第三例患有WM和淀粉样A蛋白(AA)淀粉样变性的患者。该患者出现了吸收不良、肾病综合征和体位性低血压。在直肠活检中通过免疫组织化学方法证实了AA的存在。结合之前AA淀粉样变性的病例,本报告提出了AA淀粉样变性也可能发生在WM患者中的可能性。

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