Kasabach-Merritt syndrome: clinical vs. surgical treatment

• Autor(a) principal: Moura,Regina
• Data de Publicação: 2014
• Outros Autores: Sobreira,Marcone Lima, Bertanha,Matheus, Jaldin,Rodrigo Gibin, Silva,Maria Madalena, Pereira,Lied, Takegawa,Bonifacio Katsunori, Yoshida,Winston Bonetti
• Tipo de documento: Artigo
• Idioma: eng
• Título da fonte: Jornal Vascular Brasileiro (Online)
• Texto Completo: http://old.scielo.br/scielo.php?script=sci_arttext&pid=S1677-54492014000400330
• topic: Kasabach-Merritt syndrome; amputation; corticosteroids; hemangioma

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.