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惰性系统性肥大细胞增多症的一种罕见表现及其在2019冠状病毒病大流行期间的管理;来自叙利亚的经验教训

A rare manifestation of indolent systemic mastocytosis and its management during the coronavirus disease 2019 pandemic; educational lessons from Syria.

作者信息

Slaibi Anas, Alshehabi Zuheir, Soltany Amjad, Isber Yasmin, Eid Raghad, Al-Armani Azzam, Mohanna Farah

机构信息

Department of Pathology, Cancer Research Center at Tishreen University Hospital, Latakia, Syria.

Department of Plastic and Reconstructive Surgery, Al Mouwasat University Hospital, Faculty of Medicine, Damascus University, Damascus, Syria.

出版信息

Ann Med Surg (Lond). 2021 Feb;62:293-297. doi: 10.1016/j.amsu.2021.01.062. Epub 2021 Jan 21.

Abstract

The diagnosis of indolent systemic mastocytosis can be quite a challenge due to its wide spectrum of clinical manifestations. We are reporting a case of misdiagnosed indolent systemic mastocytosis in a 41-year-old male that has been wrongly treated first as a Non-Hodgkin lymphoma. The patient had generalized lymphadenopathy and eosinophilia, which are rare manifestations of indolent systemic mastocytosis. The chronic neglected pruritus and elevated tryptase levels along with histological findings were the main clues that have led us to the final diagnosis of indolent systemic mastocytosis. Later during the coronavirus disease 2019 pandemic, the patient presented with symptoms that are common between coronavirus disease 2019 and mast cell activation syndrome. Coronavirus disease 2019 was confirmed with a polymerase chain reaction test. There were no clear guidelines for managing a case like ours, so our management plan was based on the latest recommendations published at that time.

摘要

惰性系统性肥大细胞增多症的诊断颇具挑战,因其临床表现范围广泛。我们报告一例41岁男性惰性系统性肥大细胞增多症误诊病例,该患者最初被误诊为非霍奇金淋巴瘤并接受了错误治疗。患者有全身淋巴结肿大和嗜酸性粒细胞增多,这在惰性系统性肥大细胞增多症中是罕见表现。慢性被忽视的瘙痒、升高的类胰蛋白酶水平以及组织学检查结果是最终确诊惰性系统性肥大细胞增多症的主要线索。后来在2019冠状病毒病大流行期间,该患者出现了2019冠状病毒病和肥大细胞活化综合征共有的症状。通过聚合酶链反应检测确诊为2019冠状病毒病。当时尚无针对我们这类病例的明确管理指南,因此我们的管理计划基于当时发布的最新建议。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/470a/7846895/8548076e0702/gr1.jpg

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