Fauchald Trym, Blomberg Bjørn, Reikvam Håkon
Faculty of Medicine, University of Bergen, 5007 Bergen, Norway.
Department of Clinical Science, University of Bergen, 5007 Bergen, Norway.
J Clin Med. 2023 Aug 18;12(16):5366. doi: 10.3390/jcm12165366.
Hemophagocytic lymphohistiocytosis (HLH) is a condition of immune dysregulation and hyperinflammation, leading to organ failure and death. Malignancy, autoimmune conditions, and infections, including Mycobacterium tuberculosis (TB), are all considered triggers of HLH. The aim of this study was to review all reported cases of TB-associated HLH in English literature, and to summarize the epidemiology, diagnostics, treatment, and mortality in patients with concomitant HLH and TB. A systematic review of described cases with TB-associated HLH, via a structured literature search in the medical database PubMed, is presented. Additional articles were included through cross-referencing with existing review articles. Articles were reviewed based on a predetermined set of criteria. A total of 116 patients with TB-associated HLH were identified with a male:female ratio of about 3:2. The age at presentation ranged from 12 days to 83 years. Malignancy, autoimmunity, and renal failure were the most common comorbid conditions. Most patients received both tuberculostatic and specific immunomodulating treatment, which was associated with a 66% (48/73) survival rate compared to 56% (15/27) in those receiving only tuberculostatic treatment, and 0% (0/13) in those receiving only immunomodulating treatment. The survival rate was 55% overall. The overlapping presentation between disseminated TB and HLH poses challenging diagnostics and may delay diagnosis and treatment, leading to increased mortality. TB should be considered as a potential trigger of HLH; clinicians' knowledge and awareness of this may result in the appropriate investigations needed to ensure diagnosis and proper treatment.
噬血细胞性淋巴组织细胞增生症（HLH）是一种免疫失调和过度炎症状态，可导致器官衰竭和死亡。恶性肿瘤、自身免疫性疾病以及包括结核分枝杆菌（TB）在内的感染，均被视为HLH的触发因素。本研究的目的是回顾英文文献中所有报道的结核相关HLH病例，并总结合并HLH和TB患者的流行病学、诊断、治疗及死亡率。本文通过在医学数据库PubMed中进行结构化文献检索，对已描述的结核相关HLH病例进行了系统综述。通过与现有综述文章交叉引用纳入了其他文章。根据预先设定的标准对文章进行了评审。共确定了116例结核相关HLH患者，男女比例约为3:2。发病年龄范围为12天至83岁。恶性肿瘤、自身免疫性疾病和肾衰竭是最常见的合并症。大多数患者接受了抗结核和特异性免疫调节治疗，其生存率为66%（48/73），而仅接受抗结核治疗的患者生存率为56%（15/27），仅接受免疫调节治疗的患者生存率为0%（0/13）。总体生存率为55%。播散性TB和HLH之间的重叠表现给诊断带来了挑战，可能会延迟诊断和治疗，导致死亡率增加。TB应被视为HLH的潜在触发因素；临床医生对此的认识可能会促使进行适当的检查以确保诊断和正确治疗。
