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中华实验和临床感染病杂志(电子版) ›› 2024, Vol. 18 ›› Issue (02) : 122 -127. doi: 10.3877/cma.j.issn.1674-1358.2024.02.010

病例报告

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
王丹妮1, 罗华婷1, 蒲霞1, 黄文祥1,()   
  1. 1. 400010 重庆,重庆医科大学附属第一医院老年病科
  • 收稿日期:2023-10-20 出版日期:2024-04-15
  • 通信作者: 黄文祥
  • 基金资助:
    2022年重庆市自然科学基金面上项目(No. CSTB2022NSCQ-MSX0092)

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

Danni Wang1, Huating Luo1, Xia Pu1, Wenxiang Huang1,()   

  1. 1. Department of Geriatrics, The First Affiliated Hospital of Chongqing Medical University, Chongqing 400010, China
  • Received:2023-10-20 Published:2024-04-15
  • Corresponding author: Wenxiang Huang
引用本文:

王丹妮, 罗华婷, 蒲霞, 黄文祥. 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[J]. 中华实验和临床感染病杂志(电子版), 2024, 18(02): 122-127.

Danni Wang, Huating Luo, Xia Pu, Wenxiang Huang. A case of secondary hemophagocytic lymphohistiocytosis caused by disseminated histoplasmosis and literatures review[J]. Chinese Journal of Experimental and Clinical Infectious Diseases(Electronic Edition), 2024, 18(02): 122-127.

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 near-complete 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.

图1 2023年2月27日患者胸腹部增强CT注:A、B:双肺散在分布实性与磨玻璃结节,双肺支气管血管束增多,走行正常;C、D:肝脏及脾脏增大
图2 骨髓涂片(瑞氏染色,1 000 ×)注:骨髓巨噬细胞中呈现数量不等、大小较为一致的圆形或卵圆形孢子(黑色箭头):直径3~5 μm,一端稍圆,一端稍尖,胞核染成紫红色、圆形或半圆形,约占胞体的1/3~1/2,孢子外围绕一圈未染色的空晕,形似荚膜(实为胞壁收缩所致)
图3 患者胸部增强CT注:A、B:2023年3月9日:双肺广泛实变影,并散在斑片状磨玻璃影,双侧胸腔少量积液,双肺下叶部分肺组织外压性不张,纵膈数个肿大淋巴结。C、D:2023年3月15日:双肺广泛炎症和间质性实变较2023年3月9日减少,双侧胸腔积液较前增多。E、F:2023年3月22日:双侧间质性炎症和胸腔积液较2023年3月15日明显减少,纵膈淋巴结较前稍缩小。G、H:2023年6月6日:双肺散在炎症较2023年3月22日病灶明显吸收减少,双侧胸腔积液消失
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