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一位老年患者继发性噬血细胞性淋巴组织细胞增生症的诊断困境

A Diagnostic Dilemma of Secondary Hemophagocytosis Lymphohistiocytosis in an Elderly Patient.

作者信息

Mulkareddy Vani, Bhalla Varun, Garg Ankit

机构信息

Internal Medicine, Rochester General Hospital, Rochester, USA.

Internal Medicine, Kasturba Medical College, Manipal, IND.

出版信息

Cureus. 2020 Jun 7;12(6):e8482. doi: 10.7759/cureus.8482.

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a rapidly progressive fatal condition. Although well described in the pediatric population, cases of secondary HLH are seen in adolescents and young adults. In the elderly, HLH has been shown to have a poor prognosis. Owing to its varied presentation and multisystemic involvement, diagnosis is often delayed. Due to its high mortality, prompt diagnosis and treatment are crucial. Here we present a case of secondary HLH in a 69-year-old male, who presented with fever for one week. Initial laboratory workup revealed a bicytopenia and elevated creatinine. He was initially treated with broad-spectrum antibiotics; however, a comprehensive infectious workup was negative. CT scan of the abdomen revealed splenomegaly. Further investigations revealed an elevated ferritin and triglycerides. Due to the constellation of findings, he was started on corticosteroids for concerns of HLH. Bone marrow biopsy was obtained, which revealed dysplastic changes and hemophagocytosis, consistent with HLH. This case highlights the diagnostic challenge and prognosis of HLH in the elderly population, suggesting that diagnosis and treatment should not be delayed for histological confirmation.

摘要

噬血细胞性淋巴组织细胞增生症(HLH)是一种进展迅速的致命疾病。虽然在儿科人群中已有详细描述,但继发性HLH病例在青少年和年轻人中也可见到。在老年人中,HLH的预后较差。由于其表现多样且累及多系统,诊断往往延迟。鉴于其高死亡率,及时诊断和治疗至关重要。在此,我们报告一例69岁男性继发性HLH病例,该患者发热一周。初始实验室检查显示全血细胞减少和肌酐升高。他最初接受了广谱抗生素治疗;然而,全面的感染性检查结果为阴性。腹部CT扫描显示脾肿大。进一步检查发现铁蛋白和甘油三酯升高。鉴于一系列检查结果,出于对HLH的担忧,他开始接受皮质类固醇治疗。进行了骨髓活检,结果显示发育异常改变和噬血细胞现象,符合HLH。该病例突出了老年人HLH的诊断挑战和预后情况,提示不应因等待组织学确诊而延迟诊断和治疗。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/682a/7336584/2da5bc00bf87/cureus-0012-00000008482-i01.jpg

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