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妊娠合并T细胞淋巴瘤的噬血细胞性淋巴组织细胞增生症:揭开诊断难题

Hemophagocytic Lymphohistiocytosis Associated With T-cell Lymphoma in Pregnancy: Diagnostic Conundrum Unveiled.

作者信息

Siddik Nor Azlina, Abd Rahim Siti Nur Hidayah, Jamaluddin Jazlan, Yuhana Muhamad Yazli

机构信息

Family Medicine, Klinik Kesihatan Jinjang, Kuala Lumpur, MYS.

Family Medicine, Klinik Kesihatan Taman Ehsan, Kuala Lumpur, MYS.

出版信息

Cureus. 2024 Aug 5;16(8):e66170. doi: 10.7759/cureus.66170. eCollection 2024 Aug.

Abstract

Hemophagocytic lymphohistiocytosis (HLH) during pregnancy is a rare and often misdiagnosed disease. The clinical manifestations are non-specific, contributing to a high maternal mortality rate. This case report details the presentation of a 31-year-old pregnant woman with high-grade fever initially treated as an infection-related condition. The diagnostic challenge arose from the rarity of HLH, its variable clinical presentation, and the lack of specificity in clinical and laboratory findings. Despite numerous tests and escalation of therapies, the patient, unfortunately, succumbed to HLH associated with T-cell lymphoma. This case report aims to raise awareness of HLH, emphasizing its challenging definition. Malignancy-associated HLH is not uncommon, and early identification and treatment are paramount to prevent progressive tissue damage, organ failure, and mortality. The atypical presentation of HLH as a clinical manifestation of T-cell lymphoma underscores the need for vigilance in diagnosing this potentially fatal syndrome.

摘要

妊娠期间的噬血细胞性淋巴组织细胞增生症(HLH)是一种罕见且常被误诊的疾病。其临床表现不具特异性,导致孕产妇死亡率很高。本病例报告详细介绍了一名31岁高热孕妇的情况,该孕妇最初被当作感染相关疾病进行治疗。HLH的罕见性、临床表现的多样性以及临床和实验室检查结果缺乏特异性,给诊断带来了挑战。尽管进行了大量检查并加强了治疗,但不幸的是,该患者死于与T细胞淋巴瘤相关的HLH。本病例报告旨在提高对HLH的认识,强调其定义具有挑战性。恶性肿瘤相关的HLH并不罕见,早期识别和治疗对于预防进行性组织损伤、器官衰竭和死亡至关重要。HLH作为T细胞淋巴瘤的临床表现的非典型呈现凸显了在诊断这种潜在致命综合征时保持警惕的必要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/fa32/11372851/daeb0e4f5aab/cureus-0016-00000066170-i01.jpg

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本文引用的文献

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Adult haemophagocytic syndrome.成人噬血细胞性淋巴组织细胞增生症。
Lancet. 2014 Apr 26;383(9927):1503-1516. doi: 10.1016/S0140-6736(13)61048-X. Epub 2013 Nov 27.

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