An uncommon cause of distributive shock: Lessons from two consecutive cases of idiopathic systemic capillary leak syndrome (Clarkson's disease)

2015 ◽  
Vol 34 (4) ◽  
pp. 251-253 ◽  
Author(s):  
Stanislas Ledochowski ◽  
Marie Freichet ◽  
Cyril Prieur ◽  
Arnaud Friggeri ◽  
Jean-Christophe Lega
Keyword(s):  
Capillary Leak Syndrome ◽  
Capillary Leak ◽  
Systemic Capillary Leak Syndrome ◽  
Distributive Shock ◽  
Clarkson’S Disease

Capillary leak syndrome (CLS) from rituximab therapy of lymphoma

2020 ◽  
pp. 1-3
Keyword(s):  
Chemotherapy Regimen ◽  
Capillary Leak Syndrome ◽  
Plasma Extravasation ◽  
Capillary Leak ◽  
Radiological Findings ◽  
Systemic Capillary Leak Syndrome ◽  
Drug Induced ◽  
Multi Agent ◽  
Clarkson’S Disease

Capillary Leak Syndome (CLS) is characterized by plasma extravasation into the interstitium with resultant hypotension, anasarca, hemoconcentration, and hypoalbuminemia in the absence of albuminuria. Initially reported in Clarkson’s disease (systemic capillary leak syndrome, SCLS), CLS has been observed in multiple disease settings, the most common being sepsis. In Oncology, CLS has been reported more often as a complication from therapy, and less often from malignancy. In this case study, we documented clinical manifestation, laboratory features and radiological findings of CLS from rituximab therapy when employed in combination with a multi-agent chemotherapy regimen (EPOCH-R). Differentiating drug-induced CLS from sepsis, which presents with the same clinical features, is important in avoiding further exposure to rituximab, which could be fatal to the patient.

scholarly journals Systemic Capillary Leak Syndrome (Clarkson’s Disease) as a Complication of Anti–Programmed Death 1 Immunotherapy

2019 ◽  
Vol 14 (6) ◽  
pp. e131-e132 ◽  
Author(s):  
Céline Lescure ◽  
Alain Lescoat ◽  
Alexandre Salé ◽  
Yann Bazin ◽  
Loïg Duvergé ◽  
...  
Keyword(s):  
Capillary Leak Syndrome ◽  
Capillary Leak ◽  
Systemic Capillary Leak Syndrome ◽  
Programmed Death ◽  
Programmed Death 1 ◽  
Clarkson’S Disease

scholarly journals Diagnosis of Systemic Capillary Leak Syndrome in a Young Child Treated with Intravenous Immunoglobulin in the Acute Phase

2017 ◽  
Vol 07 (02) ◽  
pp. 094-096 ◽  
Author(s):  
Ashley Bjorklund ◽  
Gwenyth Fisher ◽  
Anna Sofi Asmundsson
Keyword(s):  
Intravenous Immunoglobulin ◽  
Acute Phase ◽  
Clinical Course ◽  
Pediatric Intensive Care ◽  
Capillary Leak Syndrome ◽  
Capillary Leak ◽  
Systemic Capillary Leak Syndrome ◽  
Acute Therapy ◽  
Vasopressor Support ◽  
Distributive Shock

AbstractSystemic capillary leak syndrome (SCLS) is a potentially life-threatening disorder characterized by distributive shock, hypoalbuminemia, and hemoconcentration. It is exceedingly rare in children with less than 20 cases reported to date. The underlying cause for this syndrome remains largely unknown and acute treatment has remained mainly supportive. Prophylaxis with intravenous immunoglobulin (IVIG) has been shown to successfully prevent further episodes in both adults and children. We present a case of a 2-year-old previously healthy male admitted to the pediatric intensive care unit with a clinical course consistent with SCLS. His shock was refractory to aggressive fluid and vasopressor support. Reversal of SCLS with IVIG given in the acute phase had been described in three adult subjects, and for this reason, the decision was made to administer IVIG. Within an hour of administration, hemodynamics stabilized and vasopressor support could be weaned. He has had no further episodes on prophylactic infusions of IVIG. Although the exact mechanism of IVIG in SCLS is unknown, it has proven to be an effective and safe prophylactic therapy, and in our patient, it drastically reversed the acute capillary leak. We suggest that IVIG should be considered as acute therapy in pediatric patients with refractory shock and a clinical course suggestive of SCLS.

Systemic capillary leak syndrome or Clarkson’s disease: a case report

2009 ◽  
Vol 4 (4) ◽  
pp. 357-358 ◽  
Author(s):  
Maria De Martino ◽  
Laura Sasso ◽  
Flora Pirozzi ◽  
Domenico Bonaduce
Keyword(s):  
Case Report ◽  
Capillary Leak Syndrome ◽  
Capillary Leak ◽  
Systemic Capillary Leak Syndrome ◽  
Clarkson’S Disease

scholarly journals Hyperviscosity-related splenic infarction, gastrointestinal ischaemia–reperfusion injury and transient dysarthria in a patient with distributive shock due to idiopathic systemic capillary leak syndrome (Clarkson’s syndrome)

2020 ◽  
Vol 13 (1) ◽  
pp. e232500
Author(s):  
Jelle Alexander van Erven ◽  
Jolanda Schrama ◽  
Daan Albert Robertus Castelijn
Keyword(s):  
Splenic Infarction ◽  
Capillary Leak Syndrome ◽  
Polycythaemia Vera ◽  
Capillary Leak ◽  
Myeloproliferative Disease ◽  
Systemic Capillary Leak Syndrome ◽  
Ischaemia Reperfusion Injury ◽  
Correct Treatment ◽  
Ischaemia Reperfusion ◽  
Distributive Shock

Clarkson’s syndrome, also known as idiopathic systemic capillary leak syndrome, is characterised by vascular hyperpermeability resulting in intravascular hypovolaemia and shock. A clinician should consider the diagnosis if other causes of shock, for example, sepsis and anaphylaxis, are ruled out and concomitant hyperviscosity is not caused by a myeloproliferative disease. Here, we describe a patient presenting with severe plasma leakage and assumable blood hyperviscosity leading to splenic infarction, gastrointestinal ischaemia–reperfusion syndrome and transient dysarthria. Our patient was first suspected of polycythaemia vera and phlebotomies were performed. Awareness of this syndrome and subsequent correct treatment is essential to prevent complications and to reduce mortality. As in our patient, most patients with Clarkson’s syndrome have a monoclonal gammopathy, light-chain-type kappa. Prophylactic treatment with intravenous immunoglobulin (IVIg) is advised to prevent recurrence of capillary leak. Our patient did not suffer from another symptomatic episode after starting IVIg.

scholarly journals Idiopathic Systemic Capillary Leak Syndrome (Clarkson's Disease): The Mayo Clinic Experience

2010 ◽  
Vol 85 (10) ◽  
pp. 905-912 ◽  
Author(s):  
Prashant Kapoor ◽  
Patricia T. Greipp ◽  
Eric W. Schaefer ◽  
Sumithra J. Mandrekar ◽  
Arif H. Kamal ◽  
...  
Keyword(s):  
Mayo Clinic ◽  
Capillary Leak Syndrome ◽  
Capillary Leak ◽  
Systemic Capillary Leak Syndrome ◽  
Clinic Experience ◽  
Clarkson’S Disease

scholarly journals Clarkson’s Disease Episode or Secondary Systemic Capillary Leak-Syndrome

CHEST Journal ◽  
2021 ◽  
Vol 159 (1) ◽  
pp. 441
Author(s):  
Marc Pineton de Chambrun ◽  
Jean-Michel Constantin ◽  
Alexis Mathian ◽  
Cyril Quemeneur ◽  
Victoria Lepere ◽  
...  
Keyword(s):  
Capillary Leak Syndrome ◽  
Capillary Leak ◽  
Systemic Capillary Leak Syndrome ◽  
Disease Episode ◽  
Clarkson’S Disease
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