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- W3207000746 abstract "<h3></h3> Kaposiform haemangioendothelioma ( KHE) is a rare vascular tumor and has high mortality rate in newborns when associated with Kasabach-Merritt syndrome ( KMS) due to consumptive coagulopathy. <h3>Methods</h3> Female newborn, GA 30 w, BM 2220g, due to the threatening asphyxia, born by S.C., with respiratory distress syndrome (RDS) and extremly massive soft tissue purple tumor with swollen erythematous, inhomogenous skin. The tumor was spread over lower abdominal wall, vulva, gluteal region, whole right tight and upper part of the left tight. The baby was anemic (Hb 97 g/l), trombocytopenic ( 8x109/L) with consumptive coagulopathy (immeasurable fibrinogen). RDS was treated with exogenous surfactant and mechanical ventilation. As soon as possible she received packed red blood cell transfusion, platelet transfusion, fibronogen concentrate, INN-eptacog alfa (activated). On the ultrasound of the abdomen paralytic ileus and hemorrhagic effusions were found. Doppller ultrasound of the tumor expressed heterogenous echogenicity and hypervascular pattern.Hour by hour the tumor grew larger. In spite of all intensive treatment baby developed multiorgan failure (MOF) and died in the of 18 hours. <h3>Results</h3> Patohistologically, the tumor consists of irregular, predominantly small and slit-like vascular spaces lined with spindle endothelial cells which sometimes form nodular structure. On immunohistochemistry the spindle cells had positive reaction for CD31, CD34, D2-40 and negative reaction for GLUT-1. The finding corresponds to KHE. <h3>Conclusions</h3> We report a premature born neonate with a huge KHE associated with fulminant form of KMS and developed consumptive coagulopathy resulting in multiorgan failure and death within 18 hours." @default.
- W3207000746 created "2021-10-25" @default.
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- W3207000746 date "2021-10-01" @default.
- W3207000746 modified "2023-09-25" @default.
- W3207000746 title "141 Kaposiform haemangioendothelioma with Kasabach-Merritt phenomen in a newborn: a case report" @default.
- W3207000746 doi "https://doi.org/10.1136/archdischild-2021-europaediatrics.141" @default.
- W3207000746 hasPublicationYear "2021" @default.
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