Department of Hematology, Jiangsu Province Hospital, The First Affiliated Hospital of Nanjing Medical University, 300 Guangzhou Road, Nanjing, 210029, China.
Department of Geriatric Hematology, Jiangsu Province Hospital, The First Affiliated Hospital of Nanjing Medical University, 300 Guangzhou Road, Nanjing, 210029, China.
Int J Hematol. 2021 Feb;113(2):263-270. doi: 10.1007/s12185-020-03011-8. Epub 2020 Oct 9.
The clinical features of patients with secondary hemophagocytic lymphohistiocytosis (sHLH) complicated with capillary leak syndrome (CLS) remain controversial. The data of 259 sHLH patients were retrospectively analyzed. The clinical manifestations, laboratory findings, treatment, and prognosis of the CLS-sHLH group and non-CLS-sHLH group were compared. The levels of fibrinogen, albumin, and serum calcium in the CLS-sHLH group were lower than in the non-CLS-sHLH group, and serum triglycerides in the CLS-sHLH group were higher than in the non-CLS-sHLH group (P < 0.05). Univariate analysis showed that fibrinogen level was an independent prognostic factor in sHLH patients complicated with CLS. The median survival time was significantly shorter in patients with fibrinogen ≤ 1.3 g/L than in patients with fibrinogen > 1.3 g/L (P < 0.05). Patients with improved CLS conditions in the CLS-sHLH group had significantly increased albumin and serum calcium after treatment (P < 0.05); patients without improved conditions in the CLS-sHLH group also had significantly increased albumin after treatment (P < 0.05), but the serum calcium did not change significantly (P > 0.05). sHLH patients complicated with CLS had significantly worse prognosis than without CLS. Significant reduction in fibrinogen may be an independent prognostic factor for poor prognosis in sHLH patients complicated with CLS.
继发性噬血细胞性淋巴组织细胞增生症（sHLH）合并毛细血管渗漏综合征（CLS）患者的临床特征仍存在争议。回顾性分析了 259 例 sHLH 患者的数据。比较了 CLS-sHLH 组和非 CLS-sHLH 组的临床表现、实验室检查、治疗和预后。CLS-sHLH 组纤维蛋白原、白蛋白和血清钙水平低于非 CLS-sHLH 组，血清甘油三酯高于非 CLS-sHLH 组（P<0.05）。单因素分析显示，纤维蛋白原水平是 sHLH 合并 CLS 患者的独立预后因素。纤维蛋白原≤1.3g/L 的患者中位生存时间明显短于纤维蛋白原>1.3g/L 的患者（P<0.05）。CLS-sHLH 组 CLS 病情改善的患者治疗后白蛋白和血清钙明显升高（P<0.05）；CLS-sHLH 组病情未改善的患者治疗后白蛋白也明显升高（P<0.05），但血清钙无明显变化（P>0.05）。合并 CLS 的 sHLH 患者预后明显差于无 CLS 者。纤维蛋白原明显降低可能是 sHLH 合并 CLS 患者预后不良的独立预后因素。
