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不明原因发热中噬血细胞性淋巴组织细胞增生症的诊断:一例报告

Diagnosis of Hemophagocytic Lymphohistiocytosis in Pyrexia of Unknown Origin: A Case Report.

作者信息

Thapa Khusbu, Thapa Khusbu, Kharel Bikranta Bikram, Shrestha Shreya, Ikram Tanbir

机构信息

Kathmandu University School of Medical Sciences, Dhulikhel, Kavre, Nepal.

Department of Emergency Medicine, Lifeline Hospital, Jhapa, Nepal.

出版信息

JNMA J Nepal Med Assoc. 2024 May 31;62(274):401-403. doi: 10.31729/jnma.8626.

Abstract

A case of a 61-year male presented with chief complaints of fever for three months. Diagnosis of hemophagocytic lymphohistiocytosis was made after liver biopsy when Positron-Emission Tomography revealed multiple fluoro deoxy glucose avid lesions in the liver. The patient of this disease typically presents with fever, splenomegaly, hyperferritinemia, hypertriglyceridemia, raised liver enzymes, and marrow features suggestive of hemophagocytosis. The treatment is usually systemic corticosteroids, chemotherapy with etoposide, cyclosporine, or hematopoietic stem cell transplantation, depending on the cause. A diagnosis like hemophagocytic lymphohistiocytosis can easily be missed in developing countries with low resources like Nepal. Hence, this disease should be suspected in patients presenting with pyrexia of unknown origin by treating physicians.

摘要

一名61岁男性患者,以持续三个月发热为主诉就诊。正电子发射断层扫描显示肝脏有多个氟脱氧葡萄糖摄取增加的病灶,经肝脏活检后诊断为噬血细胞性淋巴组织细胞增生症。该病患者通常表现为发热、脾肿大、高铁蛋白血症、高甘油三酯血症、肝酶升高以及骨髓表现提示噬血细胞现象。治疗通常根据病因采用全身皮质类固醇、依托泊苷化疗、环孢素或造血干细胞移植。在尼泊尔这样资源匮乏的发展中国家,噬血细胞性淋巴组织细胞增生症这样的诊断很容易被漏诊。因此,对于不明原因发热的患者,治疗医生应怀疑此病。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/be17/11185309/d6f62bdb1fa2/JNMA-62-274-401-g1.jpg

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