BJH - volume 6, issue 1, march 2015
K. De Man MD, F. Van Ryckeghem MD, J. Dierick MD, B. Leus MD, V. Van Hende MD, M. Schurgers MD, H. Hannon MD
Summary
Systemic capillary leak syndrome is a potentially fatal disorder characterised by transient but severe hypotension, resulting in vascular collapse and shock, in combination with extreme hemoconcentration and anasarca oedema accompanied by a monoclonal gammopathy of unknown significance. We describe a case of Clarkson’s disease, complicated with severe hemoconcentration leading to splenic infarction and pulmonary oedema treated with ultrafiltration. The pathogenesis of systemic capillary leak syndrome remains unknown. We determined the serum concentration of soluble mediators erythropoietin and vascular endothelial growth factor, in order to attribute their role in the underlying pathophysiology of the disease.
(BELG J HEMATOL 2015;6(1):33–6)
Read more
Top
