Vascular Endothelial Growth Factor in Systemic Capillary Leak Syndrome

Lesterhuis, W. Joost; Rennings, Alexander J.; Leenders, William P. J.; Nooteboom, A; Punt, Cornelis J.A.; Sweep, Fred C.G.J.; Pickkers, Peter; Geurts-Moespot, Anneke; Laarhoven, Hanneke W. M. van; Vlag, Johan van der; Berden, Jo H. M.; Postma, Cornelis T.; Meer, J W van der

doi:10.1016/j.amjmed.2009.01.020

Cited by 61 publications

(59 citation statements)

References 9 publications

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“…The overproduction of VEGF might be associated with SCLS, but to the best of our knowledge, few studies have monitored serum VEGF levels. Lesterhuis et al reported two cases of SCLS which showed high serum VEGF levels (9). These cases are similar to the present case in view of extraordinary high serum VEGF levels.…”

Section: T a B L E 1 L A B O R A T O R Y Da T A O N Ad Mi S S I O Nsupporting

confidence: 78%

Synchronized Changes in Serum Vascular Endothelial Growth Factor during the Clinical Course of Chronic Systemic Capillary Leak Syndrome

et al. 2010

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Section: T a B L E 1 L A B O R A T O R Y Da T A O N Ad Mi S S I O Nsupporting

confidence: 78%

Synchronized Changes in Serum Vascular Endothelial Growth Factor during the Clinical Course of Chronic Systemic Capillary Leak Syndrome

et al. 2010

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“…In agreement with individual case reports, our patients had elevated serum VEGF levels at the onset of an acute SCLS episode compared with their baseline, and in one patient VEGF returned to baseline rapidly on presentation with a severe hypotensive crisis. 10,22 Our results show that a short interval of elevated VEGF (and possibly other permeability factors) may exist in SCLS. , and anti-VEGF (bevacizumab) or equivalent concentrations of control IgG (for anti-Ang2 and bevacizumab) or BSA (for IVIG) against episodic sera from 4 to 6 patients was evaluated in the TEER assay.…”

Section: Discussionmentioning

confidence: 88%

“…7,8 Although paraprotein levels in SCLS are uniformly Ͻ 1 g/dL, recent case reports of symptom resolution after treatment of the underlying plasma cell dyscrasia and a small cohort study that reported efficacy of intravenous immunoglobulin administration for prevention of SCLS episodes have suggested a pathogenic role for the monoclonal IgG in the recurrent episodes of vascular leakage. 5,9 Although early studies that used serial measurements of infused radiolabeled albumin established the link between marked, but transient, vascular hyperpermeability and the clinical manifestations of SCLS episodes, 1,10 little is known about the molecular events leading to the episodic hyperpermeability of SCLS. The only molecular clues come from the original description by Clarkson, 1 who reported that plasma drawn during an episode from an index case induced a shock-like syndrome when injected into rats and contained heparin-precipitable protein.…”

Section: Introductionmentioning

confidence: 99%

Vascular endothelial hyperpermeability induces the clinical symptoms of Clarkson disease (the systemic capillary leak syndrome)

Xie

Ghosh

Patel

et al. 2012

Blood

172

208

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The systemic capillary leak syndrome (SCLS) is a rare disorder characterized by transient episodes of hypotensive shock and anasarca thought to arise from reversible microvascular barrier dysfunction. Although the high prevalence of a monoclonal gammopathy of unknown significance in SCLS suggests a pathogenic contribution of endogenous immunoglobulins, the mechanisms of vascular hyperpermeability remain obscure. Herein, we report clinical and molecular findings on 23 patients, the largest SCLS case series to date. Application of episodic SCLS sera, but neither the purified immunoglobulin fraction nor sera obtained from patients during remission, to human microvascular endothelial cells caused vascular endothelial cadherin internalization, disruption of interendothelial junctions, actin stress fiber formation, and increased permeability in complementary functional assays without inducing endothelial apoptosis. Intravenous immunoglobulin, one promising therapy for SCLS, mitigated the permeability effects of episodic sera. Consistent with the presence of endogenous, nonimmunoglobulin, circulating permeability factor(s) constrained to SCLS episodes, we found that vascular endothelial growth factor (VEGF) and angiopoietin 2 (Ang2), were elevated in episodic SCLS sera but not in remission sera. Ab-based inhibition of Ang2 counteracted permeability induced by episodic SCLS sera.

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“…The specific role of paraproteins in the pathogenesis of SCLS has not been defined. Elevated levels of vascular endothelial growth factor and angiopoietin 2, contraction of endothelial cells due to apoptosis during SCLS attacks, and involvement of IL-2 and other inflammatory mediators, including leukotrienes and tumor necrosis factor alpha, have been implicated in the pathophysiology of SCLS [15][16][17][18][19].…”

Section: Discussionmentioning

confidence: 99%

Systemic Capillary Leak Syndrome Presenting as Apparent Polycythemia Vera: A Critical Diagnostic Dilemma

Wu¹

2014

J Hematol

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Polycythemia vera presents with true erythrocytosis (elevated red cell mass) and an expanded plasma volume, while apparent erythrocytosis presents with plasma volume contraction and a normal red cell mass. Here we report a case with recurrent episodes of severe apparent erythrocytosis, closely mimicking polycythemia vera, due to a rare condition known as systemic capillary leak syndrome (SCLS). This syndrome typically presents with the triad of hypotension, hemocencentration and hypoalbuminemia, combined with a monoclonal serum M-protein. Empirical treatment with intravenous immune globulin (IVIG) has successfully led to resolution of symptoms and correction of hemoconcentration in several reported cases as well as in our patient's case. It is important for clinicians to differentiate true erythrocytosis from apparent erythrocytosis, and to be aware of this rare syndrome causing the latter, as aggressive phlebotomy and overtreatment with crystalloid fluids could result in serious adverse events and should be avoided.

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Vascular Endothelial Growth Factor in Systemic Capillary Leak Syndrome

Cited by 61 publications

References 9 publications

Synchronized Changes in Serum Vascular Endothelial Growth Factor during the Clinical Course of Chronic Systemic Capillary Leak Syndrome

Synchronized Changes in Serum Vascular Endothelial Growth Factor during the Clinical Course of Chronic Systemic Capillary Leak Syndrome

Vascular endothelial hyperpermeability induces the clinical symptoms of Clarkson disease (the systemic capillary leak syndrome)

Systemic Capillary Leak Syndrome Presenting as Apparent Polycythemia Vera: A Critical Diagnostic Dilemma

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