Rosado Flavia G, Rinker Elizabeth B, Plummer W D, Dupont William D, Spradlin Natalie M, Reichard Kaaren K, Kim Annette S
Department of Pathology, West Virginia University, Morgantown, West Virginia, USA.
Department of Pathology, Louisiana State University Health Sciences Center, New Orleans, Louisiana, USA.
J Clin Pathol. 2016 Sep;69(9):805-9. doi: 10.1136/jclinpath-2015-203577. Epub 2016 Feb 19.
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.
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.
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.
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.
噬血细胞性淋巴组织细胞增生症（HLH）分为儿童型（原发性）和成人型（继发性）。虽然儿童HLH已得到广泛研究，但针对成人的类似研究却很有限。本研究旨在评估HLH诊断标准以及其他临床参数在有骨髓噬血细胞证据的成人中的意义。
我们对两家机构的病理档案进行了为期10年的回顾性检索，以查找有骨髓噬血细胞现象的病例。我们纳入了那些符合当前既定HLH诊断标准的病例。对于符合纳入标准的29例病例，我们评估了临床特征、合并症、治疗方法和临床结局。使用逻辑回归和风险回归分析评估19个临床变量对死亡率结局的影响。
在可确定病因的病例中，感染性疾病是最常见的关联因素（19例中的14例，74%）。发热和铁蛋白升高是用于确立HLH的最常见标准。尽管48%的病例已开始采用HLH特异性治疗，但总体死亡率仍为61%。其余病例接受了支持性治疗和抗生素治疗。死亡率最具统计学意义的标志物是绝对中性粒细胞计数（ANC）升高，这一特征并非HLH的典型表现。
由于ANC升高与脓毒症而非HLH的不良结局相关，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/全身炎症反应综合征而非HLH。我们的结果凸显了需要定义针对成人人群的HLH诊断标准。
