目的:噬血细胞性淋巴组织细胞增生症(HLH)分为儿科(原发性)和成人(继发性)类型。虽然儿科HLH已被广泛表征,类似的成人研究是有限的。这项研究旨在评估HLH诊断标准以及其他临床参数在成人骨髓吞噬证据中的重要性。
方法:我们对两个机构的病理档案进行了为期10年的回顾性检索,以寻找骨髓吞噬作用的病例。我们纳入了符合目前建立的HLH诊断标准的病例。对于符合纳入标准的29例,我们评估了临床特征,合并症,治疗和临床结果。使用逻辑和风险回归分析评估了19个临床变量对死亡率结果的影响。
结果:对于可以确定病因的病例,传染病是最常见的关联(19人中有14人,74%).发热和铁蛋白升高是用于建立HLH的最常见的可用标准。尽管有HLH特异性治疗,总死亡率为61%。在48%的病例中已经启动。其余病例接受支持治疗和抗生素治疗。最具统计学意义的死亡率指标是中性粒细胞绝对计数(ANC)升高,HLH不典型的特征。
结论:由于ANC升高与脓毒症不良预后相关,而不是HLH,我们推测,本研究中许多符合HLH诊断标准的患者可能患有脓毒症/全身性炎症反应综合征,而非HLH.我们的结果强调了定义特定于成年人群的HLH诊断标准的必要性。
OBJECTIVE: Haemophagocytic lymphohistiocytosis (HLH) is divided into paediatric (primary) and adult (secondary) types. While paediatric-HLH has been extensively characterised, similar studies in adults are limited. This study aims to evaluate the significance of the HLH diagnostic criteria as well as other clinical parameters in adults with bone marrow evidence of haemophagocytosis.
METHODS: We conducted a 10-year retrospective search of the pathology archives of two institutions for cases with bone marrow haemophagocytosis. We included those cases that fulfilled the currently established HLH diagnostic criteria. For the 29 cases that met inclusion criteria, we assessed clinical features, co-morbidities, therapy and clinical outcome. The effect of 19 clinical variables on mortality outcomes was assessed using logistic and hazard regression analyses.
RESULTS: Of cases for which an aetiology could be identified, infectious diseases were the most common association (14 of 19, 74%). Fever and elevated ferritin were the most frequently available criteria used to establish HLH. The overall mortality rate was 61% despite HLH-specific therapy, which had been initiated in 48% of the cases. The remaining cases were treated with supportive therapy and antibiotics. The most statistically significant marker of mortality was an elevated absolute neutrophil count (ANC), a feature not typical of HLH.
CONCLUSIONS: Since elevated ANC correlates with poor outcomes in sepsis, and not HLH, we postulate that many of the patients fulfilling HLH diagnostic criteria in this study likely had sepsis/systemic inflammatory response syndrome rather than HLH. Our results highlight the need to define HLH diagnostic criteria specific to the adult population.