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以结肠狭窄为表现的原发性系统性淀粉样变性:左半结肠切除术后联合自体造血干细胞移植成功治疗:病例报告

Primary systemic amyloidosis presenting as a colonic stricture: successful treatment with left hemicolectomy followed by autologous hematopoietic stem-cell transplantation: report of a case.

作者信息

Rives S, Pera M, Rosiñol L, Vidal O, Miquel R, Solé M, García-Valdecasas J, Bladé J

机构信息

Institut Clínic de Malalties Hemato-Oncològiques, Department of Hematology and Postgraduate School of Hematology Farreras Valentí, Barcelona, Spain.

出版信息

Dis Colon Rectum. 2002 Sep;45(9):1263-6. doi: 10.1007/s10350-004-6403-x.

Abstract

Intestinal tract involvement by primary systemic amyloidosis is frequent but usually asymptomatic. Ischemic colitis caused by amyloid infiltration of wall blood vessels can occasionally be observed. We report a 62-year-old female with primary systemic amyloidosis who presented with intestinal obstruction caused by ischemic stricture of the sigmoid colon, secondary to submucosal amyloid deposition. The patient was successfully treated with surgical resection followed by high-dose chemotherapy and hematopoietic stem-cell transplantation. The clinical manifestations and differential diagnosis of gastrointestinal involvement of primary systemic amyloidosis, as well as its current treatment, are discussed.

摘要

原发性系统性淀粉样变性累及肠道很常见,但通常无症状。偶尔可观察到因壁血管淀粉样浸润引起的缺血性结肠炎。我们报告一例62岁原发性系统性淀粉样变性女性患者,因乙状结肠缺血性狭窄继发黏膜下淀粉样沉积导致肠梗阻。该患者接受手术切除,随后进行大剂量化疗和造血干细胞移植,治疗成功。本文讨论了原发性系统性淀粉样变性胃肠道受累的临床表现、鉴别诊断及其当前治疗方法。

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