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与肠道淀粉样变性相关的大量肠道出血。潜在病理过程的研究。

Massive intestinal hemorrhage associated with intestinal amyloidosis. An investigation of underlying pathologic processes.

作者信息

Kaiserling E, Kröber S

机构信息

Department of Pathology, University of Tübingen, Germany.

出版信息

Gen Diagn Pathol. 1995 Oct;141(2):147-54.

PMID:8548595
Abstract

UNLABELLED

Two cases of systemic amyloidosis with massive intestinal hemorrhage necessitating bowel resection prompted an investigation of the possible pathologic processes leading to such hemorrhage, since no conclusive information about this has been published.

METHODS

The two surgical specimens and, for comparison, one biopsy specimen and autopsy specimens from six cases of amyloidosis were investigated by various histologic techniques.

RESULTS

Massive amyloid deposition in the muscularis mucosae was noted in both surgical specimens. The source of hemorrhage was identified as being located at the border between the muscularis mucosae and the overlying rectal or colonic mucosa. In the autopsy specimens, there was patchy or linear amyloid deposition in the muscularis mucosae, but no hemorrhage.

CONCLUSIONS

Various factors could be involved in causing massive intestinal hemorrhage in systemic amyloidosis. Functional disturbances may be involved due to amyloid deposition in relation to blood vessels, lymphatics, nerves, and nerve plexuses, and, as it appeared to be the case in the two surgical specimens investigated, massive deposition in the muscularis mucosae. The reduced motility and increased rigidity of the musculature probably result in shearing forces being set up in the presence of mechanical strain (eg in coprostasis or colonoscopy) that lead to tears in the region of the muscularis mucosae and to massive hemorrhage. Intestinal hemorrhage in amyloidosis may also be related to disturbances of coagulation, which have been reported in occasional cases, and ulceration, probably resulting in some cases of ischemic colitis.

摘要

未标记

两例系统性淀粉样变性伴大量肠道出血,需行肠切除术,鉴于尚未发表关于此类出血的确凿信息,因此对导致此类出血的可能病理过程进行了调查。

方法

采用多种组织学技术对两份手术标本以及作为对照的一份活检标本和六例淀粉样变性的尸检标本进行了研究。

结果

两份手术标本均发现黏膜肌层有大量淀粉样物质沉积。出血源位于黏膜肌层与覆盖其上的直肠或结肠黏膜之间的边界处。在尸检标本中,黏膜肌层有散在或线状淀粉样物质沉积,但无出血。

结论

系统性淀粉样变性导致大量肠道出血可能涉及多种因素。由于淀粉样物质沉积于血管、淋巴管、神经和神经丛,以及如在两份研究的手术标本中所见的黏膜肌层大量沉积,可能涉及功能障碍。肌肉组织运动性降低和僵硬增加,可能在存在机械性应变(如便秘或结肠镜检查时)时产生剪切力,导致黏膜肌层区域撕裂并引起大量出血。淀粉样变性中的肠道出血也可能与凝血功能障碍有关,偶尔有病例报道,还可能与溃疡有关,在某些情况下可能导致缺血性结肠炎。

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