The disease is characterized by episodes of transient vascular collapse, which leads to hypotensive shock and anasarca. 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. Capillary leak syndrome an overview sciencedirect topics. 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. Pdf gemcitabineinduced systemic capillary leak syndrome. Idiopathic systemic capillary leak syndrome clarkson. Pdf introduction the systemic capillary leak syndrome is a rare idiopathic disorder characterized by recurrent episodes of. If prophylactic treatment including intravenous immunoglobulin ivig is provided, diseasespecific mortality seems to decrease in idiopathic cls. After 60 days of treatment he was dismissed from the clinic. Fluid management is a critical part of the treatment of capillary leak syndrome. Adult subjects with systemic capillary leak syndrome scls present with acute and recurrent episodes of vascular. 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. Treatment of the systemic capillary leak syndrome with terbutaline and theophylline. 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.
He was able to return to his previous occupation and reached the same level of. 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. Initial symptoms may include fatigue, nausea, abdominal pain, extreme thirst, and sudden increase in body weight. Compartment syndrome as a result of systemic capillary leak. To the editor the systemic capillary leak syndrome scls is a rare disease of unknown etiology characterized by transient episodes of distributive shock. 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. A 56yearold woman presented with anasarca, hypoalbuminaemia and hypotension following cycle 3 day 1 of adjuvant gemcitabine for stage ii pancreatic cancer.
In 1960, dr bayard clarkson described a patient who experienced sporadic bouts of hypovolemia, hypotension, and edema. Background the systemic capillary leak syndrome is a highly rare disorder of unknown etiology. Scls is characterized by hemoconcentation and hypoalbuminemia caused by reversible plasma extravasation. Described as increased endovascular or microvascular permeability, cls is known to occur in a variety of disease states. Systemic capillary leak syndrome associated with a rare abdominal. We studied clinical disease characteristics, serum cytokine profiles, and treatment modalities in a cohort of children with documented scls. 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. Capillary leak syndrome synonyms, capillary leak syndrome. 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.
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. A systemic capillary leak syndrome clarkson syndrome in a. Idiopathic systemic capillary leak syndrome clarkson disease jaci. Vascular endothelial growth factor levels were elevated at 707 pgml reference range. Idiopathic systemic capillary leak syndrome in children. Previous treatment of this potentially devastating. 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. Bone marrow bm histiocystosis in association with the administration of granulocytemacrophage colony stimulating factor gmcsf is a rare phenomenon with few published cases.
Idiopathic systemic capillary leak syndrome scls is a rare disorder characterized by recurrent episodes of hypotensive shock caused by. Spontaneous resolution of symptoms is uncommon without treatment. Idiopathic systemic capillary leak syndrome iscls is a rare disorder characterized by episodes of severe hypotension, hypoalbuminemia, and hemoconcentration. 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. It is associated with hemoconcentration, hypoalbuminemia, and generalized edema. 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. Jcm free fulltext systemic capillary leak syndrome. 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. The disease can be idiopathic clarkson syndrome or secondary to other diseases and treatments. Systemic capillary leak syndrome nord national organization. Systemic capillary leak syndrome scls is a rare disorder characterized by acute. 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. 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.
Cureus capillary leak syndrome aggravated by influenza type. Vascular endothelial growth factor levels were elevated at 707 pgml reference. The systemic capillary leak syndrome is a highly rare disorder of unknown etiology. 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.
This results in a sharp drop in blood pressure that, if not treated, can lead to organ failure and death. Capillary leak syndrome in the pediatric population. To describe a single case of systemic capillary leak syndrome scls with a rare complication of compartment syndrome. Capillary leak syndrome secondary to influenza a virus in an adult has only been welldocumented once before in a 2016 case report. Lifethreatening capillary leak syndrome after gcsf. Attacks often last several days and require emergency care. 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. Idiopathic systemic capillary leak syndrome uptodate. 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. 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. Rapid improvement of lifethreatening capillary leak syndrome. A case of capillary leak syndrome with black tar heroin.
Capillary leak syndrome cls is a severe complication of allogeneic stem cell transplantation sct characterized by weight gain, generalized edema, hypotension, and hypoalbuminemia. Although most widely reported in the setting of sepsis and septic shock, following traumatic injury cls is also frequently seen. 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. 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. 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.
Serum protein electrophoresis showed the presence of an igg kappa paraprotein with an estimated density of 3 gl. Capillary leak syndrome article about capillary leak. In summary, the incidence of capillary leak syndrome varies from common to very rare, depending on the disease or drug in question. Capillary leak syndrome, pathogenesis, treatment, continuous blood purification 1. Systemic capillary leak syndrome symptoms and causes mayo. 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 pdf gelmek learn how to. Systemic capillary leak syndrome in a patient receiving. 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. Systemic capillary leak syndrome scls is a condition in which fluid and proteins leak out of tiny blood vessels, into surrounding tissues. 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. Largely considered a component of the systemic inflammatory response syndrome sirs, capillary leak syndrome cls is poorly defined. Idiopathic systemic capillary leak syndrome clarksons. Idiopathic systemic capillary leak syndrome in children american.
Systemic capillary leak syndrome scls is characterized by sudden shock with diffuse oedema sparing the lungs. Patients with severe capillary leak syndrome are likely to cluster in the intensive care unit, and thus, practitioners who frequently. Pdf systemic capillary leak syndrome clarkson syndrome in. Capillary leak syndrome in trauma advances in surgery. Find out information about capillary leak syndrome. There is currently no cure for systemic capillary leak syndrome scls. We report a case of capillary leak syndrome in a 37yearold female pbpc donor who received gcsf 900. The enigmatic systemic capillary leak syndrome scls named for dr clarkson. Systemic capillary leak syndrome genetic and rare diseases nih. 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. Risk factors for cls are the use of gcsf as well as the burden of pretransplant chemotherapy.
Vascular endothelial hyperpermeability induces the clinical. We report the case of a patient with idiopathic systemic capillary leak syndrome who developed an. The disease can occur in cancer patients and effective therapeutic strategies have not been established yet. Treatment of the systemic capillary leak syndrome with. During attacks of iscls, profound derangements of the vascular endothelium develop, resulting in leakage of plasma and proteins into the interstitial compartment. This can result in dangerously low blood pressure hypotensison, hypoalbuminemia, and a decrease in plasma volume hemoconcentration. 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. 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. If you have problems viewing pdf files, download the latest version of adobe. Granulocytemacrophage colonystimulating factorassociated. Systemic capillary leak syndrome associated with a rare. Currently, about 50 cases have been reported with a 5. The chronic form of iscls is extremely rare with only a few cases reported in the literature.
A collection of disease information resources and questions answered by our. Expansion of the interstitial space causes pleural. Early prediction of capillary leak syndrome in infants after. 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. Due to the temporal nature of presentation, suspicion for gemcitabineinduced capillary leak syndrome was included in the differential diagnosis. Hypotension, shock, and acute kidney injury aki often dominate the initial clinical picture. Capillary leak syndrome with pulmonary edema jama internal.
Capillary leak syndrome radiology reference article. Systemic capillary leak syndrome annals of allergy, asthma. 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. Med any combination of signs and symptoms that are indicative of a particular disease or disorder syndrome, mr.
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. This can result in dangerously low blood pressure hypotension, hypoalbuminemia, and a decrease in plasma volume hemoconcentration. Synonyms for capillary leak syndrome in free thesaurus. 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. Systemic capillary leak syndrome scls is a rare disease characterized by shock caused by capillary hyperpermeability. Gemcitabineinduced chronic systemic capillary leak syndrome. Secondary capillary leak syndrome related to pemetrexed. For the first time, we describe a case of scls evolving over 20 years and culminating in a late and fatal myeloma with amyloidosis. Jul 31, 2017 there is currently no cure for systemic capillary leak syndrome scls. 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. High dose intravenous immunoglobulin therapy of the systemic. There is no established treatment for iscls and management is mainly supportive directed towards correction of intravascular volume depletion.
Gemcitabineinduced systemic capillary leak syndrome pdf. Idiopathic systemic capillary leak syndrome misdiagnosed and treated as polycythemia vera. These conditions and factors are sources of secondary capillary leak syndrome. 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. Mechanistic classification of the systemic capillary leak. Acute bilateral renal cortical necrosis in ellp hsieh, calebbarot, nikhil. Treatment for scls during an episode is mainly supportive, aiming to stabilize symptoms and prevent severe complications. To our knowledge, this is the first case of systemic capillary leak syndrome scls reported in association with oxaliplatin. The disease is characterized by episodes of transient vascular collapse, which leads to hypotensive.
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. 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. Chronic idiopathic systemic capillary leak syndrome. Systemic capillary leak syndrome, leukotrienes, and. 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. Systemic capillary leak syndrome causes a compartment syndrome of both lower legs and a forearm.
Cls incidence is around 20% among adults, and so far unknown in the pediatric population. Viewing 1 post of 1 total author posts december 12, 2018 at 12. Mortality rates 5 years after diagnosis have been reported to be 76%. Systemic capillary leak syndrome genetic and rare diseases. Scls is difficult to diagnose prospectively because the clinical criteriahypotension, hemoconcentration, low serum albuminare nonspecific and not present in all cases.
154 627 375 1177 1047 418 715 1407 705 1154 620 1645 1634 1632 507 1323 1131 938 301 1410 1374 7 615 315 179 1624 812 557 1153 679 411 1027 1512 219 703 1468 288 234 373 1400 1359 11