University of New Mexico, Albuquerque, NM, USA.
MetroHealth Medical Center, Cleveland, OH, USA.
J Investig Med High Impact Case Rep. 2020 Jan-Dec;8:2324709620906961. doi: 10.1177/2324709620906961.
Hemophagocytic lymphohistiocytosis (HLH) is a rare, aggressive, and, if not treated, fatal disorder that is characterized by excessive immune system activation. This disorder can be precipitated by different triggers including malignancies, infections, and autoimmune disorders. Diagnosis is made by fulfilling criteria that was last updated in 2004, and treatment frequently includes management of the underlying trigger but can also include chemotherapy. In this article, we report a case of HLH in a 27-year-old male, who had been diagnosed with advanced untreated HIV, who presented to the hospital with fever and generalized fatigue with no obvious etiology. Infectious workup revealed cytomegalovirus viremia, and the patient met HLH criteria with impressive hyperferritinemia of 15 432 ng/mL. The patient was started on treatment for cytomegalovirus infection that led to resolution of HLH. Our report highlights the importance of early detection of HLH in special populations, and that treating the presumptive trigger can lead to resolution of HLH.
噬血细胞性淋巴组织细胞增生症（HLH）是一种罕见的侵袭性疾病，如果不加以治疗，可致命。其特征为免疫系统过度激活。这种疾病可由多种诱因引起，包括恶性肿瘤、感染和自身免疫性疾病。诊断依据是在 2004 年最后更新的标准，治疗通常包括治疗潜在的诱因，但也可能包括化疗。本文报告了一例 27 岁男性噬血细胞性淋巴组织细胞增生症病例，该患者被诊断为未经治疗的晚期 HIV 感染，因发热和全身疲劳就诊，病因不明显。感染性检查发现巨细胞病毒血症，且患者符合 HLH 标准，表现为显著的高血清铁蛋白血症（15432ng/ml）。该患者开始接受巨细胞病毒感染治疗，随后 HLH 得到缓解。本报告强调了在特殊人群中早期发现 HLH 的重要性，以及治疗潜在诱因可导致 HLH 缓解。
