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Hemophagocytic lymphohistiocytosis is associated with Bartonella henselae infection in a patient with multiple susceptibility genes.
Annals of Clinical Microbiology and Antimicrobials ( IF 4.6 ) Pub Date : 2020-06-09 , DOI: 10.1186/s12941-020-00370-2
Tianjun Yang 1 , Qing Mei 1 , Lei Zhang 1 , Zhendong Chen 1 , Chunyan Zhu 1 , Xiaowei Fang 1 , Shike Geng 1, 2 , Aijun Pan 1, 2
Affiliation  

Adult-onset hemophagocytic lymphohistiocytosis (HLH) is a rare and life-threatening condition, which is often triggered by certain types of infection, cancer and numerous autoimmune diseases; however, of the numerous infectious triggers associated with HLH, the consequences of Bartonella henselae infection have been rarely reported. A 48-year-old female presented with a 20-day history of intermittent fever accompanied by systemic rash, fatigue, anorexia and weight loss later she developed shock and unconsciousness. Blood tests showed a reduction of leukocyte, anemia and thrombocytopenia, and pathological results of a bone marrow biopsy confirmed hemophagocytic activity. Metagenomic next-generation sequencing (mNGS) analysis of the lymph node detected the presence of B. henselae. Whole exome sequencing revealed two gene variants, STXBP2 and IRF5, in this adult patient with secondary HLH. Then, she received minocycline and rifampin combination anti-infective therapy. Intravenous immunoglobulin for 5 days followed by a high dose of methylprednisolone were also administered. The patient was successfully discharged from the intensive care unit and remained in good condition after 2 months of follow-up. mNGS served crucial roles in obtaining an etiological diagnosis, which suggested that screening for B. henselae should be considered in patients with HLH, especially those with a cat at home. In addition, the genetic defects were discovered to not only be present in primary HLH, but also in secondary HLH, even in the elderly.

中文翻译:

在具有多个易感基因的患者中,吞噬性淋巴细胞组织细胞增生症与亨氏巴尔通体感染有关。

成人发作的吞噬性淋巴细胞组织细胞增生症(HLH)是一种罕见且危及生命的疾病,通常由某些类型的感染,癌症和许多自身免疫性疾病引发;然而,在与HLH​​相关的众多感染触发因素中,很少报道过汉氏巴尔通体感染的后果。一名48岁女性,出现间歇性发烧20天,伴有全身性皮疹,疲劳,厌食和体重减轻,后来发展为休克和神志不清。血液检查显示白细胞减少,贫血和血小板减少,骨髓活检的病理结果证实了噬血细胞活性。淋巴结的超基因组下一代测序(mNGS)分析检测到了汉逊芽孢杆菌。整个外显子组测序揭示了两个基因变异体STXBP2和IRF5,在该成年继发性HLH患者中。然后,她接受了米诺环素和利福平联合抗感染治疗。静脉注射免疫球蛋白5天,然后服用高剂量的甲基强的松龙。该患者从重症监护室成功出院,并在随访2个月后保持良好状态。mNGS在获得病原学诊断中起着至关重要的作用,这建议在HLH患者(尤其是家中有猫的患者)中应考虑对汉逊酵母的筛查。另外,发现遗传缺陷不仅存在于原发性HLH中,而且也存在于继发性HLH中,即使在老年人中也是如此。静脉注射免疫球蛋白5天,然后服用高剂量的甲基强的松龙。该患者从重症监护室成功出院,并在随访2个月后保持良好状态。mNGS在获得病原学诊断中起着至关重要的作用,这建议在HLH患者(尤其是家中有猫的患者)中应考虑对汉逊酵母的筛查。另外,发现遗传缺陷不仅存在于原发性HLH中,而且也存在于继发性HLH中,即使在老年人中也是如此。静脉注射免疫球蛋白5天,然后服用高剂量的甲泼尼龙。该患者从重症监护室成功出院,并在随访2个月后保持良好状态。mNGS在获得病原学诊断中起着至关重要的作用,这建议在HLH患者(尤其是家中有猫的患者)中应考虑对汉逊酵母的筛查。另外,发现遗传缺陷不仅存在于原发性HLH中,而且也存在于继发性HLH中,即使在老年人中也是如此。HLH患者,尤其是家中有猫的患者,应考虑使用henselae。另外,发现遗传缺陷不仅存在于原发性HLH中,而且也存在于继发性HLH中,即使在老年人中也是如此。HLH患者,尤其是家中有猫的患者,应考虑使用henselae。另外,发现遗传缺陷不仅存在于原发性HLH中,而且也存在于继发性HLH中,即使在老年人中也是如此。
更新日期:2020-06-09
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