播散型荚膜组织胞浆菌病致继发性噬血细胞性淋巴组织细胞增多症一例并文献复习  

A case of secondary hemophagocytic lymphohistiocytosis caused by disseminated histoplasmosis and literatures review

作　　者：王丹妮[1] 罗华婷 蒲霞[1] 黄文祥[1] Wang Danni;Luo Huating;Pu Xia;Huang Wenxiang(Department of Geriatrics,The First Affiliated Hospital of Chongqing Medical University,Chongqing 400010,China)

机构地区：[1]重庆医科大学附属第一医院老年病科,重庆400010

出　　处：《中华实验和临床感染病杂志（电子版）》2024年第2期122-127,共6页Chinese Journal of Experimental and Clinical Infectious Diseases(Electronic Edition)

基　　金：2022年重庆市自然科学基金面上项目(No.CSTB2022NSCQ-MSX0092)。

摘　　要：目的探讨播散型荚膜组织胞浆菌病(DH)合并继发性噬血细胞性淋巴组织细胞增多症(HLH)的诊断与治疗策略。方法分析重庆医科大学附属第一医院于2023年2月26日收治的1例播散型荚膜组织胞浆菌病合并继发性噬血细胞性淋巴组织细胞增多症的67岁老年男性患者的诊疗过程,并结合相关文献进行复习。结果患者1个月前于广西壮族自治区旅游返家(重庆市)3天后出现发热、厌食症状,伴随四肢乏力和体重减轻,在病程中反复高热,表现出全血细胞减少,铁蛋白升高,NK细胞活性下降,肝脾肿大等HLH的临床特征,通过骨髓穿刺涂片镜检、外周血宏基因组下一代测序(mNGS)、胸部增强CT确诊为DH。患者接受小剂量地塞米松诱导治疗(5 mg/d静脉推注,14天后续用2.5 mg、1次/d,静脉推注7 d)和两性霉素B脂质体(起始17 mg/d,3天内逐渐加量至100 mg、1次/d静脉滴注21 d)续用伊曲康唑(200 mg、次/12 h口服)抗真菌治疗。经确诊并治疗3个月后,患者复查胸部增强CT显示病灶基本吸收。结论播散型荚膜组织胞浆菌病伴噬血细胞性淋巴组织细胞增多症患者治疗策略应以针对原发疾病为主,辅以激素诱导治疗,同时避免使用免疫抑制药物以减轻感染,从而改善患者预后。Objective To investigate the diagnostic and therapeutic strategies for disseminated histoplasmosis(DH)complicated with secondary hemophagocytic lymphohistiocytosis(HLH).Methods The diagnosis and treatment process of a 67-year-old male patient with DH complicated with secondary HLH admitted to the First Affiliated Hospital of Chongqing Medical University on February 26th,2023 were analyzed,and the relevant literatures were reviewed.Results The case exhibited symptoms of fever and anorexia for 3 days after returning Chongqing municipality from a trip to Guangxi Zhuang Autonomous Region,accompanied by fatigue and weight loss.Throughout his illness,he experienced recurrent episodes of high fever and presented with evidence of HLH,including reduced blood cells,elevated ferritin levels,decreased natural killer(NK)cell activity and hepatosplenomegaly.Diagnostic confirmation of DH was achieved through bone marrow aspirate smear microscopy,peripheral blood metagenomic next-generation sequencing(mNGS),and enhanced computed tomography(CT)of chest images.A therapeutic regimen was initiated,comprising low-dose dexamethasone induction therapy(5 mg daily intravenously for 14 days,followed by 2.5 mg daily for 7 days),in conjunction with amphotericin B liposomal(17 mg at the first day,the dose gradually increased from 17 mg daily to 100 mg daily over three days,and maintained for a subsequent 21 days),followed by a maintenance therapy with itraconazole(200 mg every 12 hours).A follow-up enhanced chest CT scan after three months of post-diagnosis and treatment indicated a nearcomplete resolution of the lesions.Conclusions For patients with DH combined with secondary HLH,the primary treatment strategy should focus on addressing the primary disease,supplemented by hormone induction therapy.The avoidance of immunosuppressive drugs to mitigate infection can significantly improve the prognosis.

关 键 词：播散型荚膜组织胞浆菌病 噬血细胞性淋巴组织细胞增多症 不明原因发热 呼吸困难 肝脾肿大 

分 类 号：R519.8[医药卫生—内科学]