Deparment of Laboratory Medicine, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China.
State Key Laboratory for Diagnosis and Treatment of Infectious Diseases, Collaborative Innovation Center for Diagnosis and Treatment of Infectious Diseases, National Clinical Research Center for Infectious Diseases, Zhejiang University School of Medicine First Affiliated Hospital, Hangzhou, China.
Front Cell Infect Microbiol. 2022 Jun 16;12:847950. doi: 10.3389/fcimb.2022.847950. eCollection 2022.
Hemophagocytic lymphohistiocytosis (HLH) secondary to infection is a rare disorder with poor outcome. Although cases of patients with human immunodeficiency virus (HIV) infection have been well documented, little study has reported in the setting of HIV seronegative. In this study, we report a case of HLH secondary to histoplasmosis in an immunocompetent patient in China and review all cases on this situation. The objective was to summary their epidemiology, clinical characteristics, diagnostic approaches, and therapeutic response. A 46-year-old male cooker presented fever, fatigue, anorexia, and weight loss. Bone marrow examination suggest fungus organism and hemophagocytosis, and further, bone marrow culture confirmed , as the etiology of HLH. The patient was successfully treated. We reviewed a total of the 13 cases (including our patient) of HLH with histoplasmosis in intact immunology patients. Twelve of the 13 patients are from endemic areas, and nine of the 12 cases are from emerging endemic areas, India and China. Three patients had sojourn history may related to the disease onset. Twelve of the 13 cases fulfilled HLH-2004 criteria. The diagnosis of infection was established by histological examination (13 of 13), culture (4 of 13), molecular method (2 of 13), and antigen or serological assays (2 of 13). Amphotericin B, posaconazole, and itraconazole show favorable activity against the fungus, seven patients used specific treatment for HLH. For analysis of outcomes, two of the 13 patients died. Our present case report and literature review show that disseminated infection with HLH in the immunocompetent population becomes increasingly common in emerging endemic areas and have high mortality. It is necessary for clinicians to improve the awareness of disease diagnosis due to the atypical population and disease presentation. Timely diagnosis and early use of antifungal agents will lead to favorable prognosis.
噬血细胞性淋巴组织细胞增生症（HLH）继发于感染是一种预后不良的罕见疾病。虽然人类免疫缺陷病毒（HIV）感染患者的病例已有充分记录，但在 HIV 血清阴性的情况下，几乎没有研究报道。在本研究中，我们报告了一例中国免疫功能正常患者继发于组织胞浆菌病的 HLH，并回顾了所有此类病例。目的是总结其流行病学、临床特征、诊断方法和治疗反应。一名 46 岁的男性厨师出现发热、疲劳、厌食和体重减轻。骨髓检查提示真菌和噬血细胞现象，进一步骨髓培养证实为 HLH 的病因。患者成功接受治疗。我们共回顾了 13 例（包括我们的患者）完整免疫患者中组织胞浆菌病继发 HLH 的病例。13 例患者中有 12 例来自流行地区，其中 9 例来自新兴流行地区，包括印度和中国。3 例患者有旅居史，可能与疾病发病有关。13 例患者中有 12 例符合 HLH-2004 标准。13 例患者中有 13 例通过组织学检查（13 例中的 13 例）、培养（13 例中的 4 例）、分子方法（13 例中的 2 例）和抗原或血清学检测（13 例中的 2 例）确定了感染的诊断。两性霉素 B、泊沙康唑和伊曲康唑对真菌具有良好的活性，7 例患者使用 HLH 的特定治疗方法。分析结局，13 例患者中有 2 例死亡。我们的病例报告和文献复习表明，新兴流行地区免疫功能正常人群中播散性感染导致 HLH 的情况越来越常见，死亡率较高。由于患者人群和疾病表现不典型，临床医生有必要提高对疾病诊断的认识。及时诊断和早期使用抗真菌药物将带来良好的预后。
