Wang M M, Qiu H X, Wang J J, Gao X, Duan L M, Tian T, Cheng W Y, Liu L L
Department of Hematology, The First Affiliated Hospital of Nanjing Medical University, Jiangsu Province Hospital, Nanjing 210029, China.
Department of Geriatric Hematology, The First Affiliated Hospital of Nanjing Medical University, Jiangsu Province Hospital, Nanjing 210029, China.
Zhonghua Xue Ye Xue Za Zhi. 2019 Jun 14;40(6):502-506. doi: 10.3760/cma.j.issn.0253-2727.2019.06.010.
To investigate the clinical characteristics of secondary hemophagocytic lymphohistiocytosis (sHLH) complicated with capillary leak syndrome (CLS) . The clinical and laboratory data of 87 sHLH patients, who were treated in our hospital between January 2015 and December 2017, were retrospectively analyzed. Depending on whether they were complicated with CLS, 21 sHLH patients were classified as the CLS-sHLH group, while 66 were classified as the non-CLS-sHLH group. The differences of clinical manifestations, laboratory tests, treatment and prognosis between the two groups were compared. There was no significant difference in the etiology of sHLH between the CLS-sHLH group and the non-CLS-sHLH group (>0.05) . The neutrophil, fibrinogen and albumin levels in the CLS-sHLH group were lower than those in the non-CLS-sHLH group, while the triacylglycerol levels were higher than those in the non-CLS-sHLH group (<0.05) . Varying degrees of edema, weight gain, hypotension, hypoproteinemia, oliguria and multiple serous effusions were observed in the CLS-sHLH group. Among them, there were 15 patients that CLS get improved, and the medial time of improvement was 7 (5-14) days. The other 6 patients did not get remission, while they died within 6-30 days. The median overall survival of the CLS-sHLH group was lower than that of the non-CLS-sHLH group (75 days vs not reached, =0.031) . There may be no correlation between the cause of sHLH and the occurrence of CLS. Severity of neutropenia, fibrinogen and albumin levels, and triglyceride levels may be accompanied for sHLH patients complicated with CLS. Patients with sHLH who complicated with CLS have a poor prognosis. Active treatment of HLH and its primary disease, reasonable fluid replacement and oxygen supply are crucial, which can effectively control disease progression.
探讨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）的临床特征。回顾性分析2015年1月至2017年12月在我院治疗的87例sHLH患者的临床和实验室资料。根据是否合并CLS，将21例sHLH患者分为CLS-sHLH组，66例分为非CLS-sHLH组。比较两组患者的临床表现、实验室检查、治疗及预后差异。CLS-sHLH组与非CLS-sHLH组sHLH的病因无显著差异（>0.05）。CLS-sHLH组中性粒细胞、纤维蛋白原和白蛋白水平低于非CLS-sHLH组，而三酰甘油水平高于非CLS-sHLH组（<0.05）。CLS-sHLH组观察到不同程度的水肿、体重增加、低血压、低蛋白血症、少尿和多浆膜腔积液。其中，15例CLS患者病情改善好转，改善的中位时间为7（514）天。另外6例未缓解，于630天内死亡。CLS-sHLH组的中位总生存期低于非CLS-sHLH组（75天对未达到，P=0.031）。sHLH的病因与CLS的发生可能无相关性。中性粒细胞减少、纤维蛋白原和白蛋白水平及甘油三酯水平的严重程度可能与合并CLS的sHLH患者相伴。合并CLS的sHLH患者预后较差。积极治疗HLH及其原发病、合理补液和供氧至关重要，可有效控制疾病进展。
