Sociedad Argentina de Hematología

Revista Hematología

 

 

 

 

 

Revista Argentina de Hematología

Abstract

Volumen:    17    # Number : 1

Publication Date :    Enero - Abril    Year:    2013

   PEDIATRÍA

Authors: Schwalb G, Cocca A, Attie M, Basack N, Aversa L.

Abstract: Vascular malformations are lesions caused by errors in embryonic development. According to the ISSVA, they are clasified as tumors or malformations. Among the former, the most common are hemangiomas. The diagnosis is based on clinical history and physical examination as well as diagnostic imaging. In venous malformations have been described thrombin generation and fibrinolysis evidenced through the increased dimeroD, fibrin degradation products and decreased fibrinogen concentration. These alterations may lead to LIC.Treatment of this disease includes the correction of vascular malformations and the abnormalities of hemostasis. The KMS describes different clinical situations and is associated with high morbidity and mortality secodary to thrombocytopenia by platelet trapping, microangiopathic hemolytic anemia and consumption of coagulation factors. Treatment includes supportive measures, antiplatelet drugs and in some cases surgery. The use of corticosteroids is reserved for patients with refractory disease or life- threatening hemangiomas. This update is performed in order to increase our knowledge of different aspects of this disease.

Key words: Vascular malformations, Kassabach- Merritt S, D dimer.

Pages : 55-59

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