Largely considered a component of the systemic inflammatory response syndrome sirs, capillary leak syndrome cls is poorly defined. Capillary leak syndrome synonyms, capillary leak syndrome. Systemic capillary leak syndrome scls is a rare but potentially fatal disorder characterized by a loss of fluid and proteins into the interstitial space leading to intravascular hypovolemia up. Granulocytemacrophage colonystimulating factorassociated.
Systemic capillary leak syndrome nord national organization. If prophylactic treatment including intravenous immunoglobulin ivig is provided, diseasespecific mortality seems to decrease in idiopathic cls. Systemic capillary leak syndrome genetic and rare diseases nih. We report a case of capillary leak syndrome in a 37yearold female pbpc donor who received gcsf 900. A case of capillary leak syndrome with black tar heroin. The major problem associated with cpb in newborns and infants is prolonged inflammation leading to capillary leak syndrome cls and organ dysfunction, resulting in higher morbidity and mortality 2, 4, 6, 7. In two patients with a diffuse abnormality of capillary permeability possibly related to circulating endotoxin, loss of plasma into the tissues capillary leak syndrome produced hypovolemic shock, generalized edema, hemoconcentration, and florid pulmonary edema. Capillary leak syndrome cls is complication occurring after stem cell transplantation sct, which consists in a loss of intravascular fluids into interstitial spaces after endothelial damage. Due to the temporal nature of presentation, suspicion for gemcitabineinduced capillary leak syndrome was included in the differential diagnosis. Compartment syndrome as a result of systemic capillary leak.
Rapid improvement of lifethreatening capillary leak syndrome. Based on the clinical features of the patients repeated presentations, a diagnosis of systemic capillary leak syndrome was made and the patient was commenced on oral theophylline. Systemic capillary leak syndrome in a patient receiving. There is no established treatment for iscls and management is mainly supportive directed towards correction of intravascular volume depletion. The disease can occur in cancer patients and effective therapeutic strategies have not been established yet. Capillary leak syndrome in trauma advances in surgery.
Pdf introduction the systemic capillary leak syndrome is a rare idiopathic disorder characterized by recurrent episodes of. Described as increased endovascular or microvascular permeability, cls is known to occur in a variety of disease states. Capillary leak syndrome, pathogenesis, treatment, continuous blood purification 1. The systemic capillary leak syndrome is a rare idiopathic disorder characterized by recurrent episodes of hypotension and hemoconcentration due to sudden transient extravasation of 10% to 70% of plasma. Patients with severe capillary leak syndrome are likely to cluster in the intensive care unit, and thus, practitioners who frequently round in the intensive care unit will encounter these patients. If you have problems viewing pdf files, download the latest version of adobe. Jcm free fulltext systemic capillary leak syndrome. Idiopathic systemic capillary leak syndrome in children american. Systemic capillary leak syndrome scls is a rare disease characterized by shock caused by capillary hyperpermeability. Capillary leak syndrome secondary to influenza a virus in an adult has only been welldocumented once before in a 2016 case report. To our knowledge, this is the first case of systemic capillary leak syndrome scls reported in association with oxaliplatin. Serum protein electrophoresis showed the presence of an igg kappa paraprotein with an estimated density of 3 gl.
Systemic capillary leak syndrome, leukotrienes, and. After 60 days of treatment he was dismissed from the clinic. Adult subjects with systemic capillary leak syndrome scls present with acute and recurrent episodes of vascular leak manifesting as severe hypotension, hypoalbuminemia, hemoconcentration, and generalized edema. Summary systemic capillary leak syndrome scls is a rare disorder with a high mortality rate, characterized by rapidly developing edema, weight gain and hypotension, hemoconcentration and hypoproteinemia this syndrome is caused by sudden, reversible capillary hyperpermeability with a rapid extravasation of plasma from the intravascular to the interstitial space even though scls has been. This can result in dangerously low blood pressure hypotensison, hypoalbuminemia, and a decrease in plasma volume hemoconcentration. Med any combination of signs and symptoms that are indicative of a particular disease or disorder syndrome, mr.
In two patients with a diffuse abnormality of capillary permeability possibly related to circulating endotoxin, loss of plasma into the tissues capillary leak syndrome produced hypovolemic shock, generalized edema, hemoconcentration, and florid. The disease can be idiopathic clarkson syndrome or secondary to other diseases and treatments. Idiopathic systemic capillary leak syndrome clarkson. Acute bilateral renal cortical necrosis in ellp hsieh, calebbarot, nikhil. Pdf systemic capillary leak syndrome clarkson syndrome in. This case report describes a probable diagnosis of capillary leak syndrome secondary to influenza a virus with clinical manifestations of metabolic acidosis, diffuse hypoperfusion, and respiratory failure. Risk factors for cls are the use of gcsf as well as the burden of pretransplant chemotherapy. We report the case of a patient with idiopathic systemic capillary leak syndrome who developed an. Mechanistic classification of the systemic capillary leak. The aim of the study was to analyze the clinical and laboratory data, treatment modalities, and mortality rate of patients and to identify contributing factors leading. He was able to return to his previous occupation and reached the same level of. The disease is characterized by episodes of transient vascular collapse, which leads to hypotensive. Capillary leak syndrome radiology reference article. Capillary leak syndrome cls is a severe complication of allogeneic stem cell transplantation sct characterized by weight gain, generalized edema, hypotension, and hypoalbuminemia.
Currently, about 50 cases have been reported with a 5. Bone marrow bm histiocystosis in association with the administration of granulocytemacrophage colony stimulating factor gmcsf is a rare phenomenon with few published cases. We studied clinical disease characteristics, serum cytokine profiles, and treatment modalities in a cohort of children with documented scls. Capillary leak syndrome with pulmonary edema jama internal. Fluid management is a critical part of the treatment of capillary leak syndrome. Capillary leak syndrome should be considered and his pulmonary symptoms can be quickly evaluated with a ct scan, which may reveal pericardial effusion, pulmonary edema, pleural effusion, or ascites in cases of capillary leak syndrome or signs of thrombus formation. Systemic capillary leak syndrome scls is a condition in which fluid and proteins leak out of tiny blood vessels, into surrounding tissues. Capillary leak syndrome article about capillary leak. Viewing 1 post of 1 total author posts december 12, 2018 at 12. Vascular endothelial growth factor levels were elevated at 707 pgml reference range. In 1960, dr bayard clarkson described a patient who experienced sporadic bouts of hypovolemia, hypotension, and edema. Vascular endothelial hyperpermeability induces the clinical.
May 28, 2019 idiopathic systemic capillary leak syndrome iscls, is a rare disorder characterized by recurrent attacks of varying severity of hypovolemic shock and generalized edema in association with hemoconcentration and hypoalbuminemia in the absence of albuminuria. Introduction capillary leak syndrome cls is a syndrome of hypoproteinemia, hypovolemic shock, acute renal ischemia in clinical manifestations the main reasons are the cell injury of capillary endothelial, increased vascular peand r. Scls is characterized by hemoconcentation and hypoalbuminemia caused by reversible plasma extravasation. Idiopathic systemic capillary leak syndrome clarksons. Early prediction of capillary leak syndrome in infants after. Idiopathic systemic capillary leak syndrome scls is a rare disorder characterized by recurrent episodes of hypotensive shock caused by. Systemic capillary leak syndrome associated with a rare.
A 56yearold woman presented with anasarca, hypoalbuminaemia and hypotension following cycle 3 day 1 of adjuvant gemcitabine for stage ii pancreatic cancer. Systemic capillary leak syndrome is a rare and life threatening disease characterized by periodic episodes of hypovolemic shock due to leakage of plasma from the intravascular to the extravascular space. Initial symptoms may include fatigue, nausea, abdominal pain, extreme thirst, and sudden increase in body weight. We report a case of oxaliplatininduced capillaryleak syndrome in a 63yearold man undergoing his 12th and final cycle of folfox for stage iii colorectal cancer. There is currently no cure for systemic capillary leak syndrome scls. Cureus capillary leak syndrome aggravated by influenza type. Capillary leak syndrome is a situation characterized by the escape of blood plasma through capillary walls, from the blood vessels to surrounding tissues, muscle compartments, organs or body cavities. Vascular endothelial growth factor levels were elevated at 707 pgml reference. Systemic capillary leak syndrome annals of allergy, asthma. Treatment of the systemic capillary leak syndrome with.
Hypotension, shock, and acute kidney injury aki often dominate the initial clinical picture. We performed medline and scopus searches of articles from 1960 to 2010 by using the search terms systemic capillary leak syndrome, idiopathic capillary leak syndrome, capillary leak, vascular leak, and vascular permeability, retrieving articles in english, french, and chinese. For the first time, we describe a case of scls evolving over 20 years and culminating in a late and fatal myeloma with amyloidosis. Scls is difficult to diagnose prospectively because the clinical criteriahypotension, hemoconcentration, low serum albuminare nonspecific and not present in all cases. In summary, the incidence of capillary leak syndrome varies from common to very rare, depending on the disease or drug in question. Systemic capillary leak syndrome scls is characterized by sudden shock with diffuse oedema sparing the lungs. Idiopathic systemic capillary leak syndrome scls, also known as clarksons disease and spontaneous periodic edema, is a potentially fatal disorder characterized by stereotypic attacksof varying intensity of hypovolemic shock and generalized edema in association with hemoconcentration as detected by an elevated hematocrit concentration and hypoalbuminemia, typically occurring in the.
Systemic capillary leak syndrome scls is a rare disorder characterized by acute. We report a case of oxaliplatininduced capillary leak syndrome in a 63yearold man undergoing his 12th and final cycle of folfox for stage iii colorectal cancer. Systemic capillary leak syndrome scls is a rare disorder with a high mortality rate, characterized by rapidly developing edema, weight gain and hypotension, hemoconcentration and hypoproteinemia. Secondary capillary leak syndrome related to pemetrexed. The association of cysteinyl leukotrienes with plasma exudation allowed the authors to form the hypothesis that rates of leukotriene excretion would be increased in the acute phase of systemic capillary leak syndrome scls and that leukotrienemodifier therapy would reduce the frequency and severity of lifethreatening episodes. Systemic capillary leak syndrome associated with a rare abdominal. Gemcitabineinduced chronic systemic capillary leak syndrome. Background the systemic capillary leak syndrome is a highly rare disorder of unknown etiology. Apr 01, 2010 capillary leak syndrome cls is a severe complication of allogeneic stem cell transplantation sct characterized by weight gain, generalized edema, hypotension, and hypoalbuminemia. This results in a sharp drop in blood pressure that, if not treated, can lead to organ failure and death. To the editor the systemic capillary leak syndrome scls is a rare disease of unknown etiology characterized by transient episodes of distributive shock. Cls incidence is around 20% among adults, and so far unknown in the pediatric population. Systemic capillary leak syndrome symptoms and causes mayo. It is associated with hemoconcentration, hypoalbuminemia, and generalized edema.
Systemic capillary leak syndrome is a rare idiopathic disorder, characterised by transient spontaneous episodes of macromolecular hyperpermeability with increased capillary leakage and pronounced shifts of plasma from the intravascular to the extravascular space. Systemic capillary leak syndrome scls is a rare disorder characterized by acute and severe recurrent attacks associated with a rapid fall in blood pressure as a result of fluid leaks from smaller vessels called capillaries. Systemic capillary leak syndrome scls is a rare disease with poor prognosis, characterized by the occurrence of mucocutaneous and visceral edema with hypotension, hemoconcentration, and unexpected hypoalbuminemia. Chronic idiopathic systemic capillary leak syndrome. Jul 31, 2017 systemic capillary leak syndrome scls is a condition in which fluid and proteins leak out of tiny blood vessels, into surrounding tissues.
Systemic capillary leak syndrome scls, or clarksons disease, or primary capillary leak syndrome, is a rare, grave and episodic medical condition observed largely in otherwise healthy individuals mostly in middle age. The disease is characterized by episodes of transient vascular collapse, which leads to hypotensive shock and anasarca. Capillary leak syndrome an overview sciencedirect topics. The chronic form of iscls is extremely rare with only a few cases reported in the literature. The capillary leak syndrome cls is characterized by a generalized increase in vascular permeability leading to the transfer of proteinrich fluid from the intravascular to the interstitial space. Idiopathic systemic capillary leak syndrome in children. Treatment of the systemic capillary leak syndrome with terbutaline and theophylline. Mar 10, 2012 systemic capillary leak syndrome is a rare idiopathic disorder, characterised by transient spontaneous episodes of macromolecular hyperpermeability with increased capillary leakage and pronounced shifts of plasma from the intravascular to the extravascular space. A systemic capillary leak syndrome clarkson syndrome in a. A collection of disease information resources and questions answered by our. Lifethreatening capillary leak syndrome after gcsf. Find out information about capillary leak syndrome. During attacks of iscls, profound derangements of the vascular endothelium develop, resulting in leakage of plasma and proteins into the interstitial compartment.
Synonyms for capillary leak syndrome in free thesaurus. Idiopathic systemic capillary leak syndrome uptodate. Idiopathic systemic capillary leak syndrome clarkson disease jaci. Treatment for scls during an episode is mainly supportive, aiming to stabilize symptoms and prevent severe complications. Jul 31, 2017 there is currently no cure for systemic capillary leak syndrome scls. Pdf gemcitabineinduced systemic capillary leak syndrome. These conditions and factors are sources of secondary capillary leak syndrome. Although most widely reported in the setting of sepsis and septic shock, following traumatic injury cls is also frequently seen. Idiopathic systemic capillary leak syndrome iscls, is a rare disorder characterized by recurrent attacks of varying severity of hypovolemic shock and generalized edema in association with hemoconcentration and hypoalbuminemia in the absence of albuminuria.
Adult subjects with systemic capillary leak syndrome scls present with acute and recurrent episodes of vascular. The enigmatic systemic capillary leak syndrome scls named for dr clarkson. Previous treatment of this potentially devastating. Systemic capillary leak syndrome genetic and rare diseases. Attacks often last several days and require emergency care. Gemcitabineinduced systemic capillary leak syndrome pdf. High dose intravenous immunoglobulin therapy of the systemic.
To describe a single case of systemic capillary leak syndrome scls with a rare complication of compartment syndrome. Systemic capillary leak syndrome is a rare disorder characterized by repeated flares of massive leakage of plasma from blood vessels into neighboring body cavities and muscles. 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. Mortality rates 5 years after diagnosis have been reported to be 76%. Idiopathic systemic capillary leak syndrome iscls is a rare disorder characterized by episodes of severe hypotension, hypoalbuminemia, and hemoconcentration. This can result in dangerously low blood pressure hypotension, hypoalbuminemia, and a decrease in plasma volume hemoconcentration. Systemic capillary leak syndrome pdf gelmek learn how to. Expansion of the interstitial space causes pleural. Patients with severe capillary leak syndrome are likely to cluster in the intensive care unit, and thus, practitioners who frequently. Jul 20, 2010 we performed medline and scopus searches of articles from 1960 to 2010 by using the search terms systemic capillary leak syndrome, idiopathic capillary leak syndrome, capillary leak, vascular leak, and vascular permeability, retrieving articles in english, french, and chinese. Idiopathic systemic capillary leak syndrome scls, also referred to as clarksons disease, is a unique syndrome characterized by an acute massive systemic leak of intravascular fluid resulting in. Capillary leak syndrome in the pediatric population. Spontaneous resolution of symptoms is uncommon without treatment.
The systemic capillary leak syndrome is a highly rare disorder of unknown etiology. Systemic capillary leak syndrome scls is an exceedingly rare, life and limbthreatening disorder characterized by acute and severe recurrent attacks featuring a rapid fall in blood pressure due to the temporary leak of plasma out of the blood circulatory system. Idiopathic systemic capillary leak syndrome misdiagnosed and treated as polycythemia vera. Maroz and weiner report a case of systemic capillary leak syndrome associated with administration of pemetrexed and characterized by peripheral and visceral edema, hypotension, hypoalbuminemia, and acute kidney injury. Systemic capillary leak syndrome causes a compartment syndrome of both lower legs and a forearm.
1447 1140 260 1177 1371 1597 357 1056 646 493 1227 549 301 1375 805 16 1557 708 774 1390 310 1327 1103 115 158 539 843 588 863 470 405