【摘要】 目的 分析异基因造血干细胞移植术（allogeneic hematopoietic stem cell transplantation，allo-HSCT）后并发毛细血管渗漏综合征（capillary leak syndrome，CLS）的发生率、危险因素和结局，并探讨其防治措施。 方法 回顾性分析2005年6月-2011年2月住院的allo-HSCT术后14例并发CLS的临床资料。 结果 CLS发生率为9.2%（14/152）。年龄、性别、诊断、HLA配型、预处理、CD34+细胞量、粒细胞集落刺激因子（granulocyte colony-stimulating factor，G-CSF）用量、植入时间均不能认定为造血干细胞移植后CLS诱发因素。 结论 HSCT术后CLS诱因尚不清楚，采用限水、减量G-CSF、使用糖皮质激素和羟乙基淀粉等措施及时治疗，有助于控制CLS。【Abstract】 Objective To study the occurrence rate, risk factors and outcomes of capillary leak syndrome (CLS) after allogeneic hematopoietic stem cell transplantation (allo-HSCT), and discuss its prevention and treatment. Methods We retrospectively analyzed the clinical records of 14 allo-HSCT recipients complicated with CLS from June 2005 to February 2011. Results Fourteen out of 152 patients developed CLS with a cumulative incidence of 9.2 %. None of the 8 clinical parameters including age, gender, underlying disease, donor type, conditioning regimen, CD34+ cell dose, granulocyte colony-stimulating factor (G-CSF) dosage, and days to neutrophil engraftment could be identified as risk factors for the occurrence of CLS. Conclusions Risk factors for CLS after allo-HSCT have not been fully established. Restriction of water intake, administration of corticosteroids and hydroxyethyl starch can be beneficial for patients with CLS.
ObjectiveTo investigate the effect of pulsed colloid infusion combined with continuous blood purification (CBP) for treatment of severe capillary leak syndrome (CLS). MethodsAccording to random principle,61 patients were divided into a control group(n=21),a CBP1 group(n=18) and a CBP2 group(n=22). All patients of three groups received routine treatment according to international guidelines 2008 for management of severe sepsis. The patients in the control group also received pulsed infusion colloid combined lasix. The patients in the CBP1 and CBP2 groups also received continuous veno-venous hemofiltration(CVVH) for 72 hours. The patients in the CBP1 group received concentrated colloid infusion combined lasix,and the patients in the CBP2 group received concentrated colloid infusion combined removing fluid. Blood gas analysis and Impedance Cardiography was performed before and 24,48 and 72 hours after therapy. The angiopoietin-2(Ang-2) was measured. Also the length of ICU stay,duration of mechanical ventilation,and death rate of patients in 28 days were observed. ResultsCompared with the control group and the CBP1 group,the length of ICU stay(days) and duration of mechanical ventilation (days) in the CBP2 group were significantly shorter(P<0.05),and the death rate in 28 days was lower(P<0.05). The patients in the CBP2 group showed more reduction in the APACHEⅡ score compared with the CBP1 group after therapy(P<0.05). The oxygenation index in the CBP2 group respectively increased at 24,48 and 72 hours after therapy(P<0.05). Compared with the control group and the CBP1 group,the oxygenation index in the CBP2 group respectively increased at the same time(P<0.05). The thoracic fluid content (TFC) in the CBP2 group respectively decreased at 24,48 and 72 hours(P<0.05) after therapy,and decreased compared with the control group and the CBP1 group at the same time(P<0.05). The serum levels of Ang-2 in the CBP2 group respectively decreased at 24,48 and 72 hours after therapy(P<0.05),and decreased compared with the control group and the CBP1 group at the same time(P<0.05). ConclusionPulsed colloid infusion combined with continuous blood purification can reduce the severity of capillary leak and improves the outcome of patients with severe sepsis.
ObjectiveTo evaluate the effect of intravenous immunoglobulin (IVIG) on prognosis of patients with idiopathic systemic capillary leakage syndrome (ISCLS). MethodsCase reports and case series related to IVIG on prognosis of ISCLS were electronically searched from the PubMed, CNKI and WanFang Data databases from inception to December 31, 2021. Two researchers screened literature and extracted the data independently, then, prognostic data were analyzed. ResultsA total of 143 case reports (175 patients) and 5 case series (169 patients) were included. About 75% of patients had monoclonal gamma globulin, most of those were IgG κ type. A total of 40 patients received prophylaxis with IVIG, most of whom received a high dose (2 g/kg) of IVIG per month. The 5-year and 10-year survival rates of ISCLS patients receiving IVIG secondary prevention treatment were 96% and 72%, respectively, significantly better than the rates of 66% and 43% in the group without IVIG. The median number of acute episodes per year was 0 (0-20) in the group receiving secondary prevention with IVIG and 2 (1-16) in the group not receiving IVIG. ConclusionHigh-dose (2g/kg) IVIG can improve the long-term survival of ISCLS patients, but efficacy of IVIG in acute episodes is unclear.