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因卡萨巴奇-梅里特综合征导致的下消化道出血对西罗莫司表现出显著反应。

Lower Gastrointestinal Bleeding Because of Kasabach-Merritt Syndrome Showing an Impressive Response to Sirolimus.

作者信息

Pérez Jorge Sandelis, Cintrón Dahima, Deyá-Quinquilla Alexandra, Molina Josselyn, Pagán Verónica Beyley, Vargas-Otero Pedro, Rivera Enid, Tirado-Gómez Maribel, Pacheco Eileen, Costas Pablo

机构信息

Internal Medicine Department, University of Puerto Rico-Medical Sciences Campus, San Juan, Puerto Rico.

Internal Medicine/Pediatrics Residency, University of Puerto Rico-Medical Sciences Campus, San Juan, Puerto Rico.

出版信息

ACG Case Rep J. 2022 Jun 9;9(6):e00688. doi: 10.14309/crj.0000000000000688. eCollection 2022 Jun.

Abstract

Kasabach-Merritt syndrome is a rare but life-threatening disease in which a rapidly growing vascular tumor induces localized intravascular coagulation, causing thrombocytopenia, microangiopathic hemolytic anemia, and consumption coagulopathy. It presents mainly in infants and young children. We present an adult with recurrent and severe lower gastrointestinal bleeding due to Kasabach-Merritt syndrome, treated successfully with sirolimus after multiple other failed interventions.

摘要

卡萨巴赫-梅里特综合征是一种罕见但危及生命的疾病,其中快速生长的血管肿瘤诱发局部血管内凝血,导致血小板减少、微血管病性溶血性贫血和消耗性凝血病。它主要发生于婴幼儿。我们报告一例因卡萨巴赫-梅里特综合征导致反复严重下消化道出血的成人患者,在其他多种干预措施失败后,使用西罗莫司成功治愈。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8dd5/9208868/b7a5c349b5c4/ac9-9-e00688-g001.jpg

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