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

Reversible posterior leukoencephalopathy syndrome (RPLS) is noted by a reversible syndrome of headache, altered mental function, seizures, and loss of vision associated with findings indicating predominantly posterior leukoen-cephalopathy on imaging studies. Reversible vasospasm and brain capillary leak syndrome are usually suggested to the pathophysiologic mechanism of RPLS. We report 6 patients with RPLS, which showed peculiar findings in the MRA.
Brain* ; Capillary Leak Syndrome ; Headache ; Humans ; Leukoencephalopathies* ; Posterior Leukoencephalopathy Syndrome ; Seizures

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

Systemic capillary leak syndrome (SCLS) is a condition that's caused by the shift of fluid and protein from the intravascular space to the interstitial space as a result of repetitive episodes of capillary hyperpermeability. The pathogenesis of SCLS is still unclear, but there's recently been a report showing this syndrome in association with monoclonal gammopathy. This syndrome can be a fatal disease because cardiovascular collapse can occur in the initial capillary leak phase. Although theophylline, diuretics, terbutaline, steroids, calcium antagonist, Ginkgo biloba extracts and plasmapheresis have been suggested as medication, none of them have been proven to be effective. Considering that this disease is self-limiting, conservative treatment in the acute phase is believed to be very important. Because hypoalbuminemia is very a common manifestation of SCLS, Pentastarch, which has a higher molecular weight than albumin, could be efficient to prevent cardiovascular collapse. We used 10% Pentastarch during the acute SCLS attacks of 2 patients and the patients both showed a dramatic response. Pentastarch may be helpful to treat SCLS in its initial capillary leak phase by the elevating blood pressure, and this might contribute to somewhat decreasing the acute mortality of SCLS.
Adult ; Capillary Leak Syndrome/diagnosis/*drug therapy/etiology ; Capillary Permeability ; Female ; Hetastarch/*therapeutic use ; Humans ; Plasma Substitutes/*therapeutic use

Gemcitabine is an effective newly developed chemotherapeutic agent, which is increasingly being used to treat non-small cell lung, ovarian and breast cancers. Pulmonary toxicity is usually self-limiting mild dyspnea, bronchospasm, but severe pulmonary toxicity is rarely reported. Herein, we report drug induced interstitial lung disease associated with gemcitabine treatment. High resolution computerized tomogram (HRCT) showed an increased ground glass opacity and thickened septal lines. The patient showed a rapid good response with prednisolone treatment.
Breast ; Bronchial Spasm ; Capillary Leak Syndrome ; Dyspnea ; Glass ; Humans ; Lung ; Lung Diseases, Interstitial* ; Prednisolone

Systemic capillary leak syndrome (SCLS) is a rare devastating condition that is caused by unexplained marked capillary hyperpermeability, resulting in hypovolemic shock, hemoconcentration, and hypoproteinemia. Most patients have prodromal symptoms such as non-specific gastrointestinal symptoms, myalgia, or dizziness. In the current case, we observed the patient with recurrent SCLS show perivascular lymphocytic infiltration on skin biopsy and eosinophilic infiltrates on random biopsies from duodenum to colon. This finding might be due to distinct eosinophilic enterocolitis or secondary to SCLS. However, there has been rare data for the prevention of the recurrent attack of SCLS, although the mortality is high. Therefore, we report a case of frequently relapsing SCLS responding to the corticosteroid therapy.
Biopsy ; Capillaries ; Capillary Leak Syndrome ; Colon ; Dizziness ; Duodenum ; Enterocolitis ; Eosinophils ; Humans ; Hypoproteinemia ; Prodromal Symptoms ; Shock ; Skin

Systemic capillary leak syndrome(SCLS) is a rare disorder of unknown etiology, which is characterized by recurrent attacks of hypotension, hemoconcentration, and hypoalbuminemia. Urinary or enteric loss of protein is not demonstrated. It is often associated with monoclonal gammopathy, but does not manifest multiple myeloma. Since Clarkson et al. described the first case in a 34- year-old woman, about 50 cases have been reported in the literature. However, most of the cases were of adult age, and the mean age of onset in the reported cases was 42.6 years. In literature review, we could refer only one pediatric case of SCLC by Foeldvari et al. in 1995. We report another pediatric case of SCLS.
Adult ; Age of Onset ; Capillaries ; Capillary Leak Syndrome* ; Child* ; Female ; Humans ; Hypoalbuminemia ; Hypotension ; Multiple Myeloma ; Paraproteinemias

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

OBJECTIVETo study the risk factors for capillary leak syndrome (CLS) in neonates.

METHODSThe clinical data of 52 neonates with CLS (case group) were retrospectively reviewed. Fifty hospitalized neonates without CLS were used as the control group. The possible factors for the development of CLS were identified by univariate analysis. The independent risk factors for CLS were determined by multivariate logistic regression analysis.

RESULTSThe univariate analysis showed that the incidences of hyperglycemia, respiratory distress syndrome (RDS), sepsis and cold injury syndrome in the case group were significantly higher than those in the control group (P<0.05). The logistic regression analysis showed that sepsis (OR=5.004, P=0.001), RDS (OR=3.880, P=0.013) and cold injury syndrome (OR=3.207, P=0.023) were the independent risk factors for the development of CLS.

CONCLUSIONSRDS, sepsis and cold injury syndrome are independent risk factors for CLS in neonates. Hyperglycemia may be associated with the development of CLS.

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*