Capillary leak syndrome (CLS) is a clinical syndrome characterized by impairment of vascular endothelial barrier function, increased vascular permeability, and reversible systemic edema. It is one of the early fatal complications after hematopoietic stem cell transplantation. So far, the exact pathogenesis of CLS has not been elucidated, and the diagnostic criteria and treatment methods have not been unified. At present, it is believed that the fundamental cause of CLS is hypercytokinemia, and the core factor is high permeability of vascular endothelial cells. According to the clinical manifestations, the natural course of CLS can be divided into prodrome, leakage and recovery stages. As far as treatment is concerned, symptomatic and supportive treatment is dominant according to different characteristics of each stage. In this review, the pathogenesis, clinical manifestations, diagnosis and treatment of hematopoietic stem cell transplant-associated CLS were briefly summarized.
Humans ; Capillary Leak Syndrome/diagnosis* ; Endothelial Cells ; Hematopoietic Stem Cell Transplantation/adverse effects*

Objective: To investigate the clinical characteristics of secondary hemophagocytic lymphohistiocytosis (sHLH) complicated with capillary leak syndrome (CLS) . Methods: 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. Results: There was no significant difference in the etiology of sHLH between the CLS-sHLH group and the non-CLS-sHLH group (P>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 (P<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, P=0.031) . Conclusions: 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.
Capillary Leak Syndrome ; Fibrinogen ; Humans ; Lymphohistiocytosis, Hemophagocytic ; Prognosis ; Retrospective Studies

Capillary Leak Syndrome ; complications ; Cholestasis, Intrahepatic ; complications ; Humans ; Mucocutaneous Lymph Node Syndrome ; complications

OBJECTIVETo investigate the risk factors for capillary leak syndrome (CLS) in children with malignant hematologic diseases.

METHODSThirty children with hematological malignancies complicated with CLS were analyzed with multiple logistic regression analysis.

RESULTSAt the test level of 0.05, hypoxemia and septicemia were found to significantly correlate with CLS in these children, and the number of white blood cells before CLS and severe bone marrow suppression were near the test level.

CONCLUSIONHypoxemia and septicemia are risk factors for CLS in children with malignant hematologic diseases.

Capillary Leak Syndrome ; complications ; Child ; Hematologic Neoplasms ; complications ; Humans ; Hypoxia ; complications ; Leukocyte Count ; Risk Factors ; Sepsis ; complications

A male patient undergoing extracorporeal ultrasound lithotripsy developed the symptoms of dyspnea, low blood pressure, palpitations, chest tightness, and sweating, and a clinical diagnosis of pulmonary capillary leak and hypovolemic shock was made. Pulse indicator continuous cardiac output (PiCCO) technique was used for resuscitation according to the measurements of extravascular lung water index (EVLWI) and global end-diastolic volume index (GEDI). The patient showed low levels of cardiac output (CO) and GEDI with a peak EVLWI of 32 ml/kg and profuse pink and thin sputum overflow from the trachea. The high ventilator support parameters failed to correct low oxygen saturation. Restricted fluid infusion was used to reduce pulmonary edema. Colloidal solution was given when GEDI was below 500 ml/m(2), and the volume and fluid infusion rate were reduced for a GEDI higher than 500 ml/m(2). Pulmonary edema was gradually reduced after the treatments with improvement of lactic acid level and liver and kidney functions. Vasopressors were withdrawn 6 days later, mechanical ventilation was discontinued 10 days later, and tracheal intubation was removed 25 days later, after which the patient was discharged. In the treatment of the patient, PiCCO monitoring played an important role.
Adult ; Capillary Leak Syndrome ; complications ; therapy ; Fluid Therapy ; Humans ; Lithotripsy ; adverse effects ; Male ; Pulmonary Edema ; complications ; therapy ; Shock ; complications ; therapy

Systemic capillary leak syndrome (SCLS) is very rare and lethal disease and only 150 cases have been reported after the first publication of its report in 1960 by Clarkson. SCLS is characterized by hemoconcentation and hypoalbuminemia caused by reversible plasma extravasation. Its mechanism is unknown, but transient dysfunction of the endothelium is the most suspected cause and trigger of this event may cause immunologic disarrangement. After recovery of endothelial function, fluid injected during the shock period is redistributed and can cause severe pulmonary edema. SCLS should be considered in patients with acute and severe hypotension with hemoconcentration and hypoalbuminemia without obvious cardiac dysfunction. Especially we should take into account the possibility of SCLS if fluid replacement does not work or the shock state is aggravated despite aggressive fluid resuscitation and vasopressor administration. SCLS itself is a very rare disease; furthermore, SCLS that develops during well-controlled surgery is even more rare. So we report this case with review of the literature.
Anesthesia, General* ; Capillary Leak Syndrome* ; Endothelium ; Humans ; Hypoalbuminemia ; Hypotension ; Plasma ; Publications ; Pulmonary Edema ; Rare Diseases ; Resuscitation ; Shock

Systemic capillary leak syndrome (SCLS), also called Clarkson's disease is rare and life-threatening disorder of unknown etiology, which is a characteristic triad of hypovolemic shock, hemoconcentration, and hypoalbuminemia. Unexplained capillary leakage from the intravascular to the interstitial space, which has been estimated up to 70% of the intravascular volume, is the proposed mechanism. Because the pathogenesis is unknown, it is diagnosed clinically after exclusion of other diseases that cause systemic capillary leak and no efficacious pharmacological treatment has been clearly established. The mortality rate ranges from 30% to 76%. In Korea, four cases of SCLS (5 cases in adult and 1 case in child) were reported by 2012. We describe a case of severe SCLS that suddenly occurred and rapidly progressed during pylorus preserving pancreaticoduodenectomy and review the literature.
Adult ; Capillaries ; Capillary Leak Syndrome* ; Humans ; Hypoalbuminemia ; Korea ; Mortality ; Pancreaticoduodenectomy* ; Pylorus ; Shock

Systemic capillary leak syndrome is a rare disease characterized by life-threatening attacks of reversible plasma extravasation and vascular collapse accompanied by hypotension, hemoconcentration, and hypoalbuminemia. A 36-year-old woman was admitted to this hospital with a fever, along with symptoms consistent with an upper respiratory tract infection and hypotension. Initial laboratory tests revealed several abnormal findings, including an elevated leukocyte count and hematocrit, hypoalbuminemia, and acute renal failure. Here, we report a case of successful treatment of systemic capillary leak syndrome, which can be difficult to distinguish from septic shock.
Acute Kidney Injury ; Adult ; Capillary Leak Syndrome* ; Female ; Fever ; Hematocrit ; Humans ; Hypoalbuminemia ; Hypotension ; Leukocyte Count ; Plasma ; Rare Diseases ; Respiratory Tract Infections ; Shock, Septic*

OBJECTIVETo investigate the clinical features of capillary leak syndrome (CLS) in children with sepsis, and to analyze its risk factors.

METHODSClinical data of 384 children with sepsis was studied retrospectively. They included 304 cases of general sepsis, 54 cases of severe sepsis and 26 cases of septic shock, and were divided into non-CLS (n=356) and CLS groups (n=28). Univariate analysis was performed for each of the following variables: sex, age, malnutrition, anemia, coagulation disorders, white blood cell count, C-reactive protein (CRP), procalcitonin (PCT), tumor necrosis factor (TNF), interleukin (IL)-1, IL-6, blood glucose, lactic acid, Pediatric Risk of Mortality (PRISM) III score, pediatric critical illness score (PICS), severe sepsis and number of failed organs≥3. The statistically significant variables (as independent variables) were subjected to multivariate logistic regression analysis.

RESULTSThe incidence rate of CLS in children with septic shock, severe sepsis and general sepsis were 42.3%, 20.1% and 1.3%, respectively, with significant differences among them (P<0.01). There were significant differences in anemia, coagulation disorders, CRP, PCT>2 ng/mL, TNF, IL-1, IL-6, blood glucose, lactic acid, PRISM III score, PICS and number of failed organs≥3 between the non-CLS and CLS groups (P<0.05). Severe sepsis/shock and PRISM III score were the independent risk factors for CLS in children with sepsis.

CONCLUSIONSThe severity of sepsis and PRISM III score are positively correlated with the incidence of CLS in children with sepsis. Early monitoring of such factors as infection markers and blood glucose in children with severe sepsis and high PRISM III score may contribute to early diagnosis and effective intervention, thus reducing the mortality from CLS in children with sepsis.

Adolescent ; Capillary Leak Syndrome ; epidemiology ; etiology ; Child ; Child, Preschool ; Female ; Humans ; Incidence ; Infant ; Infant, Newborn ; Logistic Models ; Male ; Retrospective Studies ; Risk Factors ; Sepsis ; complications

Adolescent ; Capillary Leak Syndrome ; chemically induced ; Female ; Granulocyte Colony-Stimulating Factor ; adverse effects ; Humans ; Male ; Middle Aged