Capillary Leak Syndrome

A condition characterized by recurring episodes of fluid leaking from capillaries into extra-vascular compartments causing hematocrit to rise precipitously. If not treated, generalized vascular leak can lead to generalized EDEMA; SHOCK; cardiovascular collapse; and MULTIPLE ORGAN FAILURE.

Also Known As:

Networked: 408 relevant articles (14 outcomes, 23 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Neoplasms (Cancer)
2.	Fever (Fevers)
3.	Hypotension (Low Blood Pressure)
4.	Exanthema (Rash)
5.	Shock

Experts

1.	Druey, Kirk M: 7 articles (01/2019 - 01/2015)
2.	Xie, Zhihui: 6 articles (01/2019 - 01/2015)
3.	Pemmaraju, Naveen: 5 articles (12/2021 - 01/2019)
4.	Pastan, Ira: 5 articles (11/2020 - 01/2013)
5.	Wu, Maddalena Alessandra: 4 articles (07/2022 - 01/2018)
6.	Castelli, Antonio: 4 articles (01/2021 - 01/2018)
7.	Catena, Emanuele: 4 articles (01/2021 - 01/2018)
8.	Colombo, Riccardo: 4 articles (01/2021 - 01/2018)
9.	Fossali, Tommaso: 4 articles (01/2021 - 01/2018)
10.	Ottolina, Davide: 4 articles (01/2021 - 01/2014)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Capillary Leak Syndrome:

1.	Intravenous Immunoglobulins (IVIG)FDA Link 01/01/2019 - "Based upon the reported responses of SCLS to IVIG, we present the case of a 54-year-old man with cSCLS where ongoing treatment with IVIG resulted in a marked and sustained improvement in the signs and symptoms of the capillary leak syndrome. ." 07/01/2018 - "Following further deterioration, Idiopathic systemic capillary leak syndrome was considered and intravenous immunoglobulin administered, resulting in rapid improvement in the patient's clinical status. " 07/01/2008 - "Intravenous immunoglobulins were effective against systemic capillary leak syndrome symptoms in three patients, but their exact mechanism remains unknown. " 07/01/2008 - "The objective of this study was to report the dramatic improvement of patients with systemic capillary leak syndrome obtained with high-dose intravenous immunoglobulins. " 01/01/2019 - "Due to the similarities between OHSS and idiopathic systemic capillary leak syndrome, we offered the patient compassionate treatment with intravenous immunoglobulins. "
2.	Interleukin-2 (IL2)IBA 09/01/2001 - "The main adverse effect of IL-2 is capillary leak syndrome caused by increased capillary permeability and extravasation of fluid. " 06/01/1999 - "Treatment with intravenous recombinant human interleukin-2 (rh IL-2) is frequently accompanied by the capillary leak syndrome and disturbances of the coagulation system. " 05/01/1996 - "These results demonstrate that during treatment with IL-2 and IFN-alpha-2a there are both fluid retention and augmented filtration of fluid from the vascular to the interstitial compartment, but no indication for a capillary leak syndrome. " 12/01/1994 - "These activated neutrophils may play an important role in initiating endothelial cell damage leading to the haemodynamic toxicity and the capillary leak syndrome which is typically seen following the administration of interleukin-2." 05/01/1993 - "One patient, who commenced IL-2 4 days after BMT, died from pulmonary oedema due to the capillary leak syndrome. "
3.	Starch (Cornstarch)IBA 01/01/2021 - "To analyze the efficacy of hydroxyethyl starch (HES) combined with Ulinastatin (Uti) in the treatment of newborns with capillary leak syndrome (CLS). " 01/01/2021 - "A preliminary study of influences of hydroxyethyl starch combined with ulinastatin on degree of edema in newborns with capillary leak syndrome."
4.	urinastatin (MR 20)IBA 01/01/2021 - "To analyze the efficacy of hydroxyethyl starch (HES) combined with Ulinastatin (Uti) in the treatment of newborns with capillary leak syndrome (CLS). " 01/01/2021 - "A preliminary study of influences of hydroxyethyl starch combined with ulinastatin on degree of edema in newborns with capillary leak syndrome."
5.	CytokinesIBA 11/01/2000 - "Because it is effective in killing T cells that surround dermal vessels, cytokine release may occur and result in capillary leak syndrome. " 01/01/2021 - "Capillary-leak syndrome is strongly associated with cytokine activity states. " 01/01/2021 - "We suggest that capillary-leak syndrome is a unique adverse effect of immunotherapy, resulting from complex interactions between cellular and cytokine activation and that its expression is probably depending on inherent host immune variabilities. " 08/01/2000 - "Cardiopulmonary bypass (CPB) in neonates induces a cytokine-mediated capillary leak syndrome that can cause organ dysfunction. " 04/01/1996 - "Under further deterioration of the pulmonary function (signs of beginning ARDS) and the generalized capillary leak syndrome we started plasmapheresis 2 days after operation in order to eliminate high-molecular cytokines. "
6.	tagraxofuspIBA 01/01/2019 - "Specifically in BPDCN, tagraxofusp was highly effective with a safety profile found to be acceptable overall, with the noted occurrence of capillary leak syndrome. " 03/23/2023 - "Tagraxofusp treatment is associated with a unique toxicity, capillary leak syndrome (CLS), which can be severe but is manageable with proper patient selection and monitoring, early recognition, and directed intervention. " 01/01/2023 - "Capillary leak syndrome is an important adverse effect of tagraxofusp that requires close monitoring. " 04/01/2021 - "Capillary leak syndrome (CLS) is the most serious adverse event reported with tagraxofusp; therefore, nurses should stop tagraxofusp administration until all CLS-related symptoms have resolved. " 01/01/2020 - "The most notable toxicity of tagraxofusp-erzs is occurrence of the capillary leak syndrome, which occurs frequently at all grades, and has also been observed to be life-threatening, appropriately leading to a US Food and Drug Administration "black box" warning in the package insert. "
7.	Nitric Oxide (Nitrogen Monoxide)FDA Link 04/01/2000 - "The common mechanism for IL-2-induced multiorgan dysfunction appears to be a capillary leak syndrome directly mediated by local production of nitric oxide by cells of the monocyte-macrophage lineage. " 01/01/1997 - "Previously, we showed that nitric oxide (NO) plays a major role in the pathogenesis of IL-2-induced capillary leak syndrome in healthy or mammary adenocarcinoma-bearing C3H/HeJ mice. " 11/01/1995 - "NG-nitro-L-arginine methyl ester, an inhibitor of nitric oxide synthesis, ameliorates interleukin-2-induced capillary leak syndrome in healthy mice." 03/01/1998 - "Role of nitric oxide in IL-2 therapy-induced capillary leak syndrome." 10/01/2001 - "Clinical application of interleukin (IL)-2-based immunotherapy of cancer has been limited by a major side-effect known as 'capillary leak syndrome', resulting from nitric oxide (NO) overproduction. "
8.	AlbuminsIBA 03/01/2002 - "We diagnosed the patient as having typical systemic capillary leak syndrome (SCLS) and she improved gradually after infusion of albumin-containing fluid. " 07/01/2013 - "An elevated hematocrit in a diver should raise the suspicion for the potential development of capillary leak syndrome requiring specific treatment using albumin infusion as primary fluid replacement." 09/01/1996 - "During cardiopulmonary bypass, albumin concentrations remained significantly lower in patients with capillary leak syndrome than in those without, whereas hematocrit values were similar in both groups. " 01/01/2020 - "Our data suggests that l-lysine administration to humans might prevent SS1P-mediated kidney damage, reduce albumin loss in urine, and alleviate capillary leak syndrome." 07/01/2005 - "To quantify capillary leak syndrome, albumin escape rate was measured using 99mTc-labelled human serum albumin. "
9.	Adrenal Cortex Hormones (Corticosteroids)IBA 10/01/1999 - "Treatment with corticosteroids should be considered for prevention of attacks in the systemic capillary leak syndrome." 07/01/2000 - "This case suggests that corticoids may be useful in the management of neoplasm induced capillary leak syndrome." 04/01/2022 - "He also exhibited systemic capillary leak syndrome (SCLS), which responded rapidly to secukinumab and systemic corticosteroids. " 10/01/2009 - "Cure of gemcitabine-induced systemic capillary leak syndrome without corticosteroids." 01/01/2002 - "Denileukin diftitox is associated with capillary leak syndrome in 20 to 30% of patients, which may be ameliorated by hydration and corticosteroids. "
10.	Levofloxacin (Levaquin)FDA Link 11/01/2002 - "We report a case of serious systemic CP infection with capillary leak syndrome and cutaneus vasculitis who was treated successfully with levofloxacin."

Therapies and Procedures

1.	Mechanical Ventilators (Ventilator) 10/01/1998 - "Whereas capillary leak syndrome was associated with prolonged mechanical ventilation (p < 0.01), there was no relationship between sCD62L concentrations at baseline or at any time thereafter and number of hours on the ventilator(p > 0.1). " 01/01/2016 - "This decrease was paralleled by marked clinical stabilization of the patient including a significant improvement in hemodynamic stability and a reduced need for norepinephrine and improved respiratory function as measured by PaO2/FIO2, ventilator parameters, lung mechanics, and indirect measures of capillary leak syndrome. "
2.	Therapeutics 01/01/2020 - "This study proposes the administration of C1-inh is an effective therapy to reduce the activation and improve the clinical capillary leak syndrome." 07/05/2023 - "No pharmacological therapy has substantial evidence in the treatment of acute systemic capillary leak syndrome. " 01/01/2021 - "Case Report: Capillary Leak Syndrome With Kidney Transplant Failure Following Autologous Mesenchymal Stem Cell Therapy." 01/01/2011 - "Pulmonary capillary leak syndrome as a result of OKT-3 therapy." 11/01/2009 - "Idiopathic systemic capillary leak syndrome: novel therapy for acute attacks."
3.	Drug Tapering 06/01/1989 - "The most prominent side-effects encountered in this trial consisted of a capillary leak syndrome, myalgia and fever requiring dose reduction in half of the patients during the first cycle. " 01/01/1989 - "The most prominent side-effects encountered in this trial consisted of a capillary leak syndrome, myalgia and fever that required dose reduction during the first cycle in one-half of the patients. "
4.	Cardiopulmonary Bypass (Heart-Lung Bypass) 08/13/2005 - "Capillary leak syndrome in children with C4A-deficiency undergoing cardiac surgery with cardiopulmonary bypass: a double-blind, randomised controlled study." 08/01/2013 - "As an inflammatory reaction after cardiac surgery involving cardiopulmonary bypass (CPB), capillary leak syndrome (CLS) is associated with increased morbidity, especially in newborns and infants. " 08/01/2013 - "Early prediction of capillary leak syndrome in infants after cardiopulmonary bypass." 09/01/2010 - "Its low priming volume and blood contact surface may significantly reduce known complications related to cardiopulmonary bypass in neonates (e.g., inflammatory reaction and capillary leak syndrome)." 08/13/2005 - "Capillary leak syndrome is a life-threatening complication after cardiopulmonary bypass (CPB), with an incidence of about 4-37% in children worldwide. "
5.	Critical Care (Surgical Intensive Care) 01/01/2003 - "Capillary leak syndrome (CLS) commonly occurs in the intensive care setting. " 01/01/2013 - "The patients with severe post-trauma capillary leak syndrome (PTCLS) admitted to department of critical care medicine of the Fifth Center Hospital in Tianjin were considered as study object. " 01/01/2020 - "One day later, he developed an acute myocarditis and a systemic capillary leak syndrome needing intensive care. " 03/01/2021 - "Cytokine release syndrome (CRS) with fever, chills, acute kidney injury, hypotension and capillary leak syndrome and CAR-T related encephalopathy syndrome (CRE) with seizures and encephaledema occurred after CAR-T cell therapy, but symptoms disappeared with effective intensive care. " 03/01/1985 - "Neutrophils from patients with evidence of diffuse capillary leak syndrome had significantly depressed enzyme specific activity as compared to neutrophils from healthy controls or intensive care patients without evidence of diffuse capillary leak. "