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一名患有爱泼斯坦-巴尔病毒(EBV)血症的老年男性的噬血细胞性淋巴组织细胞增生症(HLH)

Hemophagocytic Lymphohistiocytosis (HLH) in an Elderly Male With Epstein-Barr Virus (EBV) Viremia.

作者信息

Ghias Mona, Carducci Hugo, Romero Leslie-Joy, Haris Asif, Sunzeri Lindsay

机构信息

Internal Medicine, West Virginia University, Morgantown, USA.

Internal Medicine, West Virginia University School of Medicine, Morgantown, USA.

出版信息

Cureus. 2024 Jul 11;16(7):e64336. doi: 10.7759/cureus.64336. eCollection 2024 Jul.

DOI:10.7759/cureus.64336
PMID:39087157
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11290405/
Abstract

This is a case of a 75-year-old male with a complicated past medical history who presented initially with weakness, fevers, exertional dyspnea, cough, and confusion. His initial workup revealed elevated aspartate transaminase (AST), alanine transaminase (ALT), bilirubin, and D-dimer. Right upper quadrant (RUQ) ultrasound revealed a partially contracted gallbladder with gallstones, so he underwent laparoscopic cholecystectomy. Due to worsening hyperbilirubinemia and anemia, he later underwent a liver biopsy which showed Epstein-Barr virus (EBV)-positive lymphoid infiltration. He developed anemia, thrombocytopenia, and low fibrinogen. He met the criteria for hemophagocytic lymphohistiocytosis (HLH) with 6/8 HLH-2004 criteria and an H score of 230 with a 96-98% probability of HLH. The patient was promptly treated with steroids, rituximab, and etoposide; however, the patient's health continued to deteriorate, and he expired. This case highlights the challenges of early diagnosis of HLH in the elderly patient population due to large differentials, confounding comorbidities, and the rarity of the diagnosis in this age range.

摘要

这是一例75岁男性患者,既往有复杂病史,最初表现为乏力、发热、劳力性呼吸困难、咳嗽和意识模糊。其初始检查显示天冬氨酸转氨酶(AST)、丙氨酸转氨酶(ALT)、胆红素和D-二聚体升高。右上腹(RUQ)超声显示胆囊部分收缩伴胆结石,因此他接受了腹腔镜胆囊切除术。由于高胆红素血症和贫血加重,他后来接受了肝活检,结果显示爱泼斯坦-巴尔病毒(EBV)阳性淋巴细胞浸润。他出现了贫血、血小板减少和纤维蛋白原降低。他符合噬血细胞性淋巴组织细胞增生症(HLH)的标准,满足6/8项HLH-2004标准,H评分为230,HLH的概率为96 - 98%。患者立即接受了类固醇、利妥昔单抗和依托泊苷治疗;然而,患者的健康状况持续恶化,最终死亡。该病例凸显了在老年患者群体中早期诊断HLH的挑战,原因包括鉴别诊断范围广、合并症复杂以及该年龄范围内诊断罕见。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b2d8/11290405/ae4d72342ed3/cureus-0016-00000064336-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b2d8/11290405/ae4d72342ed3/cureus-0016-00000064336-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b2d8/11290405/ae4d72342ed3/cureus-0016-00000064336-i01.jpg

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本文引用的文献

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Adult-onset Primary Hemophagocytic Lymphohistiocytosis: Reporting a Rare Case with Review of Literature.成人起病的原发性噬血细胞性淋巴组织细胞增生症:报告1例罕见病例并文献复习
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