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Jornal Vascular Brasileiro

Print version ISSN 1677-5449On-line version ISSN 1677-7301

Abstract

MOURA, Regina et al. Kasabach-Merritt syndrome: clinical vs. surgical treatment. J. vasc. bras. [online]. 2014, vol.13, n.4, pp.330-335. ISSN 1677-5449.  http://dx.doi.org/10.1590/1677-5449.0102.

Kassabach-Merritt syndrome is a combination of capillary hemangioma and thrombocytopenia that predisposes to bleeding with petechiae, ecchymosis and spontaneous bruising. Treatment is generally started with corticosteroids, interferon alpha or chemotherapy. We present the case of a child (aged 1 year and 9 months) with a giant hemangioma, from the root of the thigh to the knee, and thrombocytopenia. Treatment was started with corticosteroids, without improvement, and then intra-tumor and cutaneous bleeding appeared spontaneously. The patient's clinical condition precluded prescription of vincristine and interferon and emergency tumor resection was conducted because of extreme thrombocytopenia and bleeding. The child then began to develop sepsis with hypotension and ischemia of remnant tissues. This case presented a therapeutic challenge, which is the subject of this article.

Keywords : Kasabach-Merritt syndrome; amputation; corticosteroids; hemangioma.

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