噬血细胞性淋巴组织细胞增生症(HLH)是一种罕见的疾病,其特征是极端的免疫激活和过度的炎症。据报道,有家族性病例的患者,免疫缺陷,恶性肿瘤,干细胞移植,和病毒病因。该报告描述了一名76岁以前健康的男性中与人类疱疹病毒6(HHV-6)感染相关的获得性HLH。病人因发烧入院,发冷,和腹痛。诊断检查在影像学上显示胆囊壁增厚,关于胆囊炎。患者开始接受脓毒症治疗。进一步的临床恶化导致广泛的感染性检查。发现患者的可溶性IL-2Ra水平升高,做了骨髓活检,这揭示了HLH。脑脊液和血清中HHV-6聚合酶链反应阳性证实了病毒感染。治疗涉及高剂量类固醇的开始,依托泊苷,还有更昔洛韦.尽管有这些干预措施,患者的临床状况恶化,导致舒适措施的实施,病人最终死了.此病例强调了将HHV-6视为有免疫能力的成年人中HLH的潜在原因的重要性。从这个案子来看,我们推断,有必要提高警惕性,以迅速认识和干预这一具有挑战性的状况。
Hemophagocytic lymphohistiocytosis (HLH) is a rare disorder characterized by extreme immune activation and excessive inflammation. It has been reported in patients with familial cases, immunodeficiencies, malignancies, stem cell transplants, and viral etiologies. This report describes acquired HLH associated with Human herpesvirus-6 (HHV-6) infection in a 76-year-old previously healthy male. The patient was admitted to the hospital due to fever, chills, and abdominal pain. The diagnostic workup revealed gallbladder wall thickening on imaging, concerning for cholecystitis. The patient was started on treatment for sepsis. Further clinical deterioration led to an extensive infectious workup. The patient was found to have elevated soluble IL-2Ra levels, and a bone marrow biopsy was performed, which revealed HLH. A positive HHV-6 polymerase chain reaction in the cerebrospinal fluid and serum confirmed the viral infection. Treatment involved the initiation of high-dose steroids, etoposide, and ganciclovir. Despite these interventions, the patient\'s clinical status worsened, leading to the implementation of comfort measures, and the patient eventually died. This case underscores the importance of considering HHV-6 as a potential cause of HLH in immunocompetent adults. From this case, we infer that a heightened level of vigilance is necessary to recognize and intervene in this challenging condition promptly.