Beneficial effect of intravenous immunoglobulins on systemic capillary leak syndrome in patients with monoclonal gammopathy

2009 ◽  
Vol 37 (2) ◽  
pp. 795
Author(s):  
Marc Lambert ◽  
David Launay ◽  
Eric Hachulla ◽  
Sandrine Morell-Dubois ◽  
Viviane Queyrel ◽  
...  
Keyword(s):  
Beneficial Effect ◽  
Monoclonal Gammopathy ◽  
Intravenous Immunoglobulins ◽  
Capillary Leak Syndrome ◽  
Capillary Leak ◽  
Systemic Capillary Leak Syndrome

scholarly journals Intravenous Immunoglobulins Improve Survival in Monoclonal Gammopathy-Associated Systemic Capillary-Leak Syndrome

2017 ◽  
Vol 130 (10) ◽  
pp. 1219.e19-1219.e27 ◽  
Author(s):  
Marc Pineton de Chambrun ◽  
Marie Gousseff ◽  
Wladimir Mauhin ◽  
Jean-Christophe Lega ◽  
Marc Lambert ◽  
...  
Keyword(s):  
Monoclonal Gammopathy ◽  
Intravenous Immunoglobulins ◽  
Capillary Leak Syndrome ◽  
Capillary Leak ◽  
Systemic Capillary Leak Syndrome

scholarly journals Idiopatic Systemic Capillary Leak Syndrome Treated Successfully with High-Dose Intravenous Immunoglobulins

2015 ◽  
Vol 13 (2) ◽  
pp. 87-89
Author(s):  
Ferhat Ekinci ◽  
Utku Erdem Soyaltın ◽  
Harun Akar ◽  
Mehmet Can Ugur ◽  
Ercan Ersoy ◽  
...  
Keyword(s):  
Case Report ◽  
Literature Review ◽  
Clinical Management ◽  
Relevant Literature ◽  
Intravenous Immunoglobulins ◽  
Capillary Leak Syndrome ◽  
High Dose ◽  
Capillary Leak ◽  
Systemic Capillary Leak Syndrome

AbstractIdiopathic systemic capillary leak syndrome (ISCLS) is characterized by a triad of hypotension, hemoconcentration and hypoalbuminemia due to a shift of intravascular fluid and albumin to the extravascular area. We describe a hypovolemic patient with hemoconcentration and hypoalbuminemia who was successfully treated with high-dose intravenous immunoglobulins (IVIG). The purpose of this case report is to discuss the clinical management of idiopathic systemic capillary leak syndrome on the background of relevant literature review.

scholarly journals Consider systemic capillary leak syndrome in monoclonal gammopathy with shock

2021 ◽  
pp. 103013
Author(s):  
Ounci-Essad ◽  
Amine Bouchlarhem ◽  
Oussama Lamzouri ◽  
Ghizlane El aidouni ◽  
Leila Haddar ◽  
...  
Keyword(s):  
Monoclonal Gammopathy ◽  
Capillary Leak Syndrome ◽  
Capillary Leak ◽  
Systemic Capillary Leak Syndrome

Idiopathic systemic capillary leak syndrome (Clarkson’s disease) presenting with recurrent hypovolemic shock

2021 ◽  
Vol 20 (1) ◽  
pp. 74-77
Author(s):  
Kristen Davies ◽  
◽  
Kirsty Thomas ◽  
Lorna Barton ◽  
Chris Williams ◽  
...  
Keyword(s):  
Hypovolemic Shock ◽  
Intravenous Immunoglobulins ◽  
Capillary Leak Syndrome ◽  
High Dose ◽  
Capillary Leak ◽  
Systemic Capillary Leak Syndrome ◽  
Compartment Syndromes ◽  
Monthly Basis ◽  
History Of ◽  
Clarkson’S Disease

A 49-year old male with a past medical history of myocardial infarction and compartment syndromes requiring fasciotomies presented on five occasions with hypovolemic shock. We describe his admissions and presumptive diagnoses which required large volumes of intravenous fluids, admission to intensive care for vasopressors and renal replacement therapy. The presentations were always precipitated by a prodrome of fatigue and pre-syncopal episodes. On his last admission, a diagnosis of Idiopathic systemic capillary leak syndrome (ISCLS), also known as Clarkson’s Disease, was reached. He is currently receiving high dose intravenous immunoglobulins on a monthly basis.

High-dose intravenous immunoglobulins dramatically reverse systemic capillary leak syndrome

2008 ◽  
Vol 36 (7) ◽  
pp. 2184-2187 ◽  
Author(s):  
Marc Lambert ◽  
David Launay ◽  
Eric Hachulla ◽  
Sandrine Morell-Dubois ◽  
Vincent Soland ◽  
...  
Keyword(s):  
Intravenous Immunoglobulins ◽  
Capillary Leak Syndrome ◽  
High Dose ◽  
Capillary Leak ◽  
Systemic Capillary Leak Syndrome

scholarly journals Multiglandular Hormone Deficiency in a Patient with Systemic Capillary Leak Syndrome

2015 ◽  
Vol 2015 ◽  
pp. 1-6 ◽  
Author(s):  
Cornelia Then ◽  
Katrin Ritzel ◽  
Christa Seibold ◽  
Johannes F. E. Mann ◽  
Martin Reincke
Keyword(s):  
Hypovolemic Shock ◽  
Intravenous Immunoglobulins ◽  
Primary Hypothyroidism ◽  
Interstitial Space ◽  
Capillary Leak Syndrome ◽  
Hormone Deficiency ◽  
Capillary Leak ◽  
Hormone Binding ◽  
Systemic Capillary Leak Syndrome ◽  
Peripheral Edema

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 to the point of hypovolemic shock. We report the case of a 64-year-old man with SCLS and multiple hormone abnormalities (primary hypothyroidism, hypoadrenalism, and hypogonadism), deficiency of hormone binding globulins, and hypogammaglobulinemia. The patient was successfully treated with intravenous immunoglobulins, theophylline, and terbutaline. Strikingly, with the dissolution of peripheral edema, hormone levels improved. To our knowledge, this is the first reported case of SCLS associated with polyglandular abnormalities.

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