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Abstract
Gorham-Stout disease (GSD) is a rare disorder characterized by proliferation of anomalous microscopic veins and/or lymphatic vessels and progressive bone destruction. The real pathogenic mechanisms haven´t been explained yet. The disease does not display a clear sex predilection or inheritance pattern. The symptoms vary and depend on the sites of the body affected, localized pain associated with extensive bone loss is the most common symptom. We present a nineteen female patient with coagulopathy and bleeding as atypical presentation of GSD
References
2. Dellinger MT, Garg N, Olsen BR: View-points on vessels and vanishing bones in Gor-ham-Stout disease. Bone. 2014;63:47-52.
3. Héritier S, Le Merrer M, Jaubert F et al. Ret-rospective French nationwide survey of child-hood aggressive vascular anomalies of bone, 1988-2009. Orphanet Journal of Rare Diseas-es. 2010;5:3.
4. J. Hagendoorn, T. Yock, I. Borel Rinkes. Novel molecular pathways in Gorham disease: Impli-cations for treatment. Pediatric Blood Cancer. 2014; 61(3),401-406.
5. Dompmartin A, Acher A, Thibon P et al. Asso-ciation of Localized Intravascular Coagulopathy With Venous Malformations. Arch Dermatol. 2008;144(7):873-877.
6. E.Mazoyer, O. Enjolras, C. Laurian et al. Coag-ulation abnormalities associated with extensive venous malformations of the limbs: differentia-tion from Kasabach-Merritt syndrome. Clin Lab Haematol. 2002 Aug;24(4):243-51.
7. M. S. Pepper, N. Ferrara, L. Orci. Vascular en-dothelial growth factor (VEGF) induces plas-minogen activators and plasminogen activator inhibitor-1 in microvascular endothelial cells. Biochem Biophys Res Commun. 1991 Dec 16;181(2):902-6.
8. Michael Pepper. Role of the Matrix Metallo-proteinase and Plasminogen Activator-Plasmin Systems in Angiogenesis. Arterioescler Thromb Vasc Biol. 2001; 21:1104-1117.
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