目的在诊断噬血细胞性淋巴组织细胞增生症(HLH)患者时确定病因和临床特征,并使用病因指导的多变量模型将这些基线特征与生存相关联。
■西班牙自身免疫性疾病HLH研究组,成立于2013年,旨在收集2013年1月3日至2017年10月28日在内科诊断为HLH的成年患者。
■该队列包括151名患者(91名男性;平均年龄,51.4年)。平均随访17个月(范围,1-142个月),80例患者死亡。死亡时间至事件分析发现,肿瘤患者的生存曲线较差(P<.001),混合微生物感染(P=0.02),1例以上感染(P=0.01)和糖皮质激素单药治疗(P=0.02)。根据单变量分析,血小板小于100,000/mm3(危险比[HR],3.39;95%CI,1.37-8.40),白细胞减少症(HR,1.81;95%CI,1.01-3.23),严重低钠血症(HR,1.61;95%CI,1.02-2.54),弥散性血管内凝血(HR,1.87;95%CI,1.05-3.34),细菌感染(HR,1.99;95%CI,1.09-3.63),混合微生物感染(HR,3.42;95%CI,1.38-8.46),和2个或更多的传染性触发因素(HR,2.95;95%CI,1.43-6.08)与死亡显著相关。相比之下,外周腺病(HR,0.63;95%CI,0.40-0.98)和免疫抑制药物/静脉注射免疫球蛋白/生物疗法(HR,0.44;95%CI,0.20-0.96)对全因死亡率具有保护作用。多变量Cox比例风险回归分析确定了2个或更多个感染触发因素(HR,3.14;95%CI,1.28-7.68)是与死亡独立相关的唯一变量。
■诊断为HLH的成年患者的死亡率超过50%。超过1种微生物制剂的感染是与死亡率相关的唯一独立变量,与潜在疾病无关。流行病学概况,临床表现,和治疗管理。
UNASSIGNED: To characterize the etiologies and clinical features at diagnosis of patients with hemophagocytic lymphohistiocytosis (HLH) and correlate these baseline features with survival using an etiopathogenically guided multivariable model.
UNASSIGNED: The Spanish Group of Autoimmune Diseases HLH Study Group, formed in 2013, is aimed at collecting adult patients with HLH diagnosed in internal medicine departments between January 3, 2013, and October 28, 2017.
UNASSIGNED: The cohort consisted of 151 patients (91 men; mean age, 51.4 years). After a mean follow-up of 17 months (range, 1-142 months), 80 patients died. Time-to-event analyses for death identified a worse survival curve for patients with neoplasia (P<.001), mixed microbiological infections (P=.02), and more than 1 infection (P=.01) and glucocorticoid monotherapy (P=.02). According to univariate analyses, platelets of less than 100,000/mm3 (hazard ratio [HR], 3.39; 95% CI, 1.37-8.40), leukopenia (HR, 1.81; 95% CI, 1.01-3.23), severe hyponatremia (HR, 1.61; 95% CI, 1.02-2.54), disseminated intravascular coagulation (HR, 1.87; 95% CI, 1.05-3.34), bacterial infection (HR, 1.99; 95% CI, 1.09-3.63), mixed microbiological infections (HR, 3.42; 95% CI, 1.38-8.46), and 2 or more infectious triggers (HR, 2.95; 95% CI, 1.43-6.08) were significantly associated with death. In contrast, peripheral adenopathies (HR, 0.63; 95% CI, 0.40-0.98) and the immunosuppressive drug/intravenous immunoglobulin/biological therapies (HR, 0.44; 95% CI, 0.20-0.96) were protective against all-cause mortality. Multivariable Cox proportional hazards regression analysis identified 2 or more infectious triggers (HR, 3.14; 95% CI, 1.28-7.68) as the only variable independently associated with death.
UNASSIGNED: The mortality rate of adult patients diagnosed with HLH exceeds 50%. Infection with more than 1 microbiological agent was the only independent variable associated with mortality irrespective of the underlying disease, epidemiological profile, clinical presentation, and therapeutic management.