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首页> 外文期刊>BMC Pediatrics >Hemophagocytic lymphohistiocytosis resulting from a cytokine storm triggered by septicemia in a child with chronic granuloma disease: a case report and literature review
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Hemophagocytic lymphohistiocytosis resulting from a cytokine storm triggered by septicemia in a child with chronic granuloma disease: a case report and literature review

机译：由慢性肉芽肿疾病的儿童败血症引发的细胞因子风暴引发的血小杂细胞淋巴管激动症：案例报告和文献综述

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Hemophagocytic lymphohistiocytosis (HLH) is a rare potentially fatal illness characterized by impaired natural killer and cytotoxic T cell function. Chronic granulomatous disease (CGD) is an inherited immune deficiency caused by a defect in the nicotinamide adenine dinucleotide phosphate (NADPH) oxidase complex. CGD patients display an increased susceptibility to infection with bacteria and fungi. Repeated infections lead to an increased risk for developing HLH. The case of CGD with repeated Salmonella septicemia complicated with HLH is very rare, and the CGD mutation identified has not been reported. A 3-year-old boy was admitted to our hospital for fever, hepatosplenomegaly and pancytopenia. According to the clinical manifestations and laboratory results, hemophagocytic lymphohistiocytosis (HLH) was diagnosed. Blood and bone marrow culture confirmed septicemia due to Salmonella Typhimurium. On the basis of antiinfection treatment, methylprednisolone was used to control HLH. After treatment, the clinical symptoms and laboratory results improved. Gene analysis showed a novel hemizygous CYBB gene mutation: c.302A??G (p.H101P). Combined with a past history of recurrent infection, the child was diagnosed with HLH secondary to CGD triggered by septicemia. In case of a known (or highly suspected) CGD with a documented infection, clinical or biological features of HLH should encourage the physician to make possible to confirm or not the HLH. Therefore, to initiate the adequate treatment in association with anti-infective therapy.
机译：血小杂细胞淋巴管激菌症（HLH）是一种罕见的潜在致命的疾病，其特征是天然杀伤性受损和细胞毒性T细胞功能。慢性肉芽肿疾病（CGD）是由烟酰胺腺嘌呤二核苷酸磷酸酯（NADPH）氧化酶复合物的缺陷引起的遗传性免疫缺陷。 CGD患者显示患有细菌和真菌的感染增加的易感性。反复感染导致培养HLH的风险增加。具有重复沙门氏菌的CGD与HLH复杂的CGD是非常罕见的，并且尚未报告鉴定的CGD突变。一名3岁的男孩被录取到我们的医院发烧，肝脾肿大和PancyTopenia。根据临床表现和实验室结果，诊断出噬血细胞淋巴管胞增生症（HLH）。由于沙门氏菌伤寒血尿，血液和骨髓文化证实了败血症。在抗抗凝固处理的基础上，使用甲基己酮酮用于控制HLH。治疗后，临床症状和实验室结果改善。基因分析显示了一种新的嗜血性CybB基因突变：C.302A？>Δg（p.h101p）。结合过去的经常感染历史，儿童被诊断为由败血症引发的CGD次级的HLH。在具有记录的感染的已知（或高度可疑）CGD的情况下，HLH的临床或生物学特征应该鼓励医生能够确认或不是HLH。因此，开始与抗感染治疗结合的充分处理。

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