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Revista Paulista de Pediatria

Print version ISSN 0103-0582On-line version ISSN 1984-0462


MUKAI, Adriana de Oliveira et al. Kaposiform haemangioendothelioma and Kasabach-Merritt syndrome. Rev. paul. pediatr. [online]. 2008, vol.26, n.2, pp.192-196. ISSN 1984-0462.

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.

Keywords : haemangioendothelioma; Kasabach-Merritt syndrome; disseminated intravascular coagulation.

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