Hemangioendotelioma kaposiforme e síndrome de Kasabach-Merritt

OBJETIVO: Descrever a apresentacao e a evolucao de um caso de hemangioendotelioma kaposiforme complicado por sindrome de Kasabach-Merritt. DESCRICAO DO CASO: Lactente masculino de 45 dias, com febre, irritabilidade e choro, associados ao aparecimento de nodulacoes em tronco e membros. Evoluiu rapidamente com coagulopatia de consumo e queda do estado geral, necessitando de internacao em Unidade de Terapia Intensiva Pediatrica (UTIP). Tomografia computadorizada revelou condensacoes parenquimatosas intra e extrapulmonares compativeis com hemangiomas multiplos e ausencia de lesoes em crânio e abdome. Houve complicacao para sindrome Kasabach-Merritt em consequencia de uma hemangiomatose kaposiforme. Optado por iniciar tratamento conservador, associando terapia de suporte com concentrado de globulos vermelhos, criopreciptado, plasma fresco, concentrado de plaquetas e uso das seguintes drogas: acido tranexâmico e dexametasona. Apos 20 dias em UTIP, ja se encontrava em bom estado geral, em processo de remissao dos hemangiomas e normalizacao dos exames laboratoriais, sendo transferido para a enfermaria. Recebeu alta em tratamento com acido tranexâmico e prednisona, com acompanhamento ambulatorial e laboratorial semanal. COMENTARIOS: A doenca apresenta curso clinico rapido e grave, com necessidade de diagnostico e tratamento precoces, para melhor prognostico e sobrevida.(AU) To describe the clinical presentation of kaposiform hemangioendothelioma that was complicated by Kasabach-Merritt syndrome. CASE DESCRIPTION: A 45-day-old male infant presented fever, irritability and crying associated with the eruption of nodules in trunk and members. Soon after admission, the patient developed a consumptive coagulopathy, requiring admission in the Pediatric Intensive Care Unit (PICU). Computed tomography scans showed intra and extrapulmonary condensations suggestive of multiple thoracic hemangiomas, without the central nervous system and abdominal lesions. The diagnosis of Kasabach-Merritt syndrome in consequence of kaposiform hemagioendothelioma was established. Treatment option was conservative, including red blood cells, cryoprecipited, fresh frozen plasma and platelets transfusions associated to tranexamic acid and dexamethasone. After 20 days, patient recovered and he was discharged from PICU with significant remission of the hemangiomas. The child continued to receive tranexamic acid and prednisone at home, with weekly follow-up. COMMENTS: The disease presents quick and severe clinical course. Therefore, early diagnosis and treatment are needed to improve the outcome.(AU)