From the Senior Department of Haematology, The First Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou, Zhejiang Province, China.
QJM. 2018 Aug 1;111(8):541-547. doi: 10.1093/qjmed/hcy103.
Lymphoma-associated hemophagocytic syndrome (LAHS) is a highly fatal immune disorder. Poor prognosis is partly attributed to under diagnosis or delayed diagnosis.
Early identification of LAHS patients based on the laboratory findings could improve the outcomes.
Retrospective observational cross-sectional study.
From January 2011 to June 2016, 282 adult patients with hemophagocytosis in bone marrow were enrolled, and 114 hemophagocytic lymphohistiocytosis (HLH) patients with definite underlying cause were finally included for analysis. The HLH patients were further divided into LAHS (76 out of 114) and non-malignancy-associated HLH (38 out of 114) groups.
Compared to non-malignancy-associated HLH, LAHS patients had significantly elevated lactate dehydrogenase (LDH) levels, increased thickness of spleen, higher proportion of patients with lymphadenopathy and significantly decreased peripheral blood cell count. In multivariate logistic regression model analysis, thickness of spleen ≥5 cm (OR = 17.9, 95%CI 1.35-236.6; P = 0.028), IL-6 level ≥55.1 pg/ml (OR = 12.01, 95%CI 1.03-138.9; P = 0.047) and IL-10 level ≥425.9 pg/ml (OR = 51.18, 95%CI 2.53-1035.1; P = 0.010) were independent predictors of LAHS diagnosis. Based on the regression parameters, we established a diagnostic index with weighted risk scores of 1 assigned to thickness of spleen and IL-6 level respectively, and a score of 3 assigned to IL-10 level. A diagnostic index ≥ 2 points had the best AUC value (0.889) with 84.2% of sensitivity and 93.7% of specificity for predicting LAHS.
LAHS can be considered when HLH patients have a diagnostic index ≥2 points, so actively looking for evidence of lymphoma and effective chemotherapy may be necessary.
淋巴瘤相关噬血细胞综合征（LAHS）是一种高病死率的免疫性疾病。部分患者预后不良是由于诊断不足或延迟诊断所致。
根据实验室检查结果，早期识别 LAHS 患者，改善患者结局。
回顾性观察性横断面研究。
2011 年 1 月至 2016 年 6 月，共纳入 282 例骨髓噬血细胞患者，最终纳入 114 例明确潜在病因的噬血细胞淋巴组织细胞增生症（HLH）患者进行分析。HLH 患者进一步分为 LAHS（114 例中 76 例）和非恶性肿瘤相关性 HLH（114 例中 38 例）两组。
与非恶性肿瘤相关性 HLH 相比，LAHS 患者乳酸脱氢酶（LDH）水平显著升高，脾脏厚度增加，淋巴结肿大比例更高，外周血血细胞计数显著减少。多变量 logistic 回归模型分析显示，脾脏厚度≥5cm（OR=17.9，95%CI 1.35-236.6；P=0.028）、IL-6 水平≥55.1pg/ml（OR=12.01，95%CI 1.03-138.9；P=0.047）和 IL-10 水平≥425.9pg/ml（OR=51.18，95%CI 2.53-1035.1；P=0.010）是 LAHS 诊断的独立预测因子。基于回归参数，我们建立了一个诊断指数，其中脾脏厚度和 IL-6 水平的权重风险评分分别为 1，IL-10 水平的权重风险评分 3。诊断指数≥2 分对 LAHS 的预测具有最佳 AUC 值（0.889），其灵敏度为 84.2%，特异性为 93.7%。
当 HLH 患者的诊断指数≥2 分时，应考虑 LAHS，因此可能需要积极寻找淋巴瘤的证据和有效的化疗。
