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对有移植相关胃肠道血栓性微血管病证据的造血干细胞移植患者进行胃肠道活检的完整组织学方法。

A Complete Histologic Approach to Gastrointestinal Biopsy From Hematopoietic Stem Cell Transplant Patients With Evidence of Transplant-Associated Gastrointestinal Thrombotic Microangiopathy.

作者信息

Warren Mikako, Jodele Sonata, Dandoy Christopher, Myers Kasiani C, Wallace Gregory, Nelson Adam, El-Bietar Javier

机构信息

From the Department of Pathology and Laboratory Medicine, Children's Hospital Los Angeles, Los Angeles, California (Dr Warren); and the Division of Bone Marrow Transplant and Immune Deficiency, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio (Drs Jodele, Dandoy, Myers, Wallace, Nelson, and El-Bietar).

出版信息

Arch Pathol Lab Med. 2017 Nov;141(11):1558-1566. doi: 10.5858/arpa.2016-0599-RA. Epub 2017 Aug 10.

Abstract

CONTEXT

  • Transplant-associated thrombotic microangiopathy is a serious complication of hematopoietic stem cell transplant that may progress to multi-organ dysfunction. Transplant-associated thrombotic microangiopathy may involve the intestinal vasculature (intestinal transplant-associated thrombotic microangiopathy [iTMA]), causing patients to experience debilitating symptoms of ischemic colitis, including disproportionately severe abdominal pain and gastrointestinal bleeding, requiring heavy narcotic use and frequent transfusion support. Pathophysiology remains poorly investigated but may include endothelial damage mediated by inflammatory markers and the complement system. Endoscopy of hematopoietic stem cell transplant patients often produces biopsy samples, in which mucosal lamina propria capillaries are sufficient for an evaluation of iTMA features.

OBJECTIVE

  • To provide a detailed review of histologic features of iTMA.

DATA SOURCES

  • We conducted a systematic review of studies assessing histologic features of iTMA. Studies were identified by PubMed search and included a cohort study performed by our group.

CONCLUSIONS

  • The histologic hallmark of iTMA is endothelial cell injury that leads to hemorrhage and thrombosis of the capillaries. Histologic features include endothelial cell swelling, endothelial cell separation, perivascular mucosal hemorrhage, intraluminal schistocytes, intraluminal fibrin, intraluminal microthrombi, loss of glands, and total denudation of mucosa. Identification of features consistent with iTMA has immediate implications for clinical management that could potentially improve outcome and survival.
摘要

背景

移植相关血栓性微血管病是造血干细胞移植的一种严重并发症,可进展为多器官功能障碍。移植相关血栓性微血管病可能累及肠道血管(肠道移植相关血栓性微血管病[iTMA]),导致患者出现缺血性结肠炎的衰弱症状,包括不成比例的严重腹痛和胃肠道出血,需要大量使用麻醉剂和频繁输血支持。其病理生理学研究仍较少,但可能包括炎症标志物和补体系统介导的内皮损伤。造血干细胞移植患者的内镜检查常可获取活检样本,其中黏膜固有层毛细血管足以评估iTMA特征。

目的

对iTMA的组织学特征进行详细综述。

数据来源

我们对评估iTMA组织学特征的研究进行了系统综述。通过PubMed搜索确定研究,其中包括我们团队进行的一项队列研究。

结论

iTMA的组织学标志是内皮细胞损伤,导致毛细血管出血和血栓形成。组织学特征包括内皮细胞肿胀、内皮细胞分离、血管周围黏膜出血、管腔内裂体细胞、管腔内纤维蛋白、管腔内微血栓、腺体缺失和黏膜完全剥脱。识别与iTMA一致的特征对临床管理具有直接意义,可能改善预后和生存率。

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