141 Kaposiform haemangioendothelioma with Kasabach-Merritt phenomen in a newborn: a case report

• Published in: Archives of disease in childhood Vol. 106; no. Suppl 2; p. A60
• Main Authors: Kondža, Karmen, Škarić, Ivančica
• Format: Journal Article
• Language: English
• Published: London BMJ Publishing Group Ltd and Royal College of Paediatrics and Child Health 11-10-2021; BMJ Publishing Group LTD
• Subjects: Abdomen; Abdominal wall; Abstracts; Asphyxia; Blood transfusion; Case reports; CD34 antigen; Endothelial cells; Erythrocytes; Fibrinogen; Hemorrhage; Immunohistochemistry; Mechanical ventilation; Neonates; Newborn babies; Pediatrics; Respiratory distress syndrome; Transfusion; Tumors; Ultrasonic imaging; Ultrasound; Ventilation; Vulva
• ISSN: 0003-9888; 1468-2044
• DOI: 10.1136/archdischild-2021-europaediatrics.141

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.MethodsFemale 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.ResultsPatohistologically, 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.ConclusionsWe 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.