Naslov
Kaposiform hemangioendothelioma with Kasabach- Merritt phenomenon in a neonate: successful treatment with sirolimus

Autori
Vukšić, Iva ; Ćaleta, Tomislav ; Džepina, Petra ; Ninković, Dorotea ; Dasović- Buljević, Andrea ; Filipović-Grčić, Boris ; Benjak, Vesna ; Ozretić, David ; Petrović Gluščić, Ana ; Bilić, Ernest ; Radoš, Marko ; Sindičić Dessardo, Nada ; Grizelj, Ruža

Vrsta, podvrsta i kategorija rada
Sažeci sa skupova, sažetak, znanstveni

Izvornik
Archives of disease in childhood, 106 (2021), Suppl 2 / - , 2021, A52-A52

Skup
10th Congress of European Paediatric Association EPA/UNEPSA jointly held with 14 th Congress of Croatian Paediatric Society

Mjesto i datum
Zagreb, Hrvatska, 07.10.2021. - 09.10.2021

Vrsta sudjelovanja
Poster

Vrsta recenzije
Međunarodna recenzija

Ključne riječi
kaposiform hemangioendothelioma ; Kasabach-Merritt phenomenon ; neonate ; sirolimus

Sažetak
Kaposiform hemangioendothelioma (KHE) is extremely rare, life- threatening vascular tumor with estimated incidence of 0.071 cases per 100.000 children. It is notably associated with Kasabach- Merritt phenomenon (KMP), a condition characterised by profound thrombocytopenia, hypofibrinogenemia, and elevated markers of coagulation activation (D-dimers or fibrin degradation products). Mortality is highly associated with degree of coagulopathy. Patients diagnosed prenatally appear to have increased disease severity. Optimal therapy for KHE is not known. Oral steroids and vincristine for patients with inoperable tumors is most commonly reported, but is associated with limited response and significant side effects. Sirolimus (rapamycin), a mammalian target of rapamycin (mTOR) inhibitor, is currently being tested in a prospective phase II clinical trial. Here we present a full-term newborn prenatally diagnosed with giant vascular tumor, affecting the lateral neck.

Izvorni jezik
Engleski

Znanstvena područja
Kliničke medicinske znanosti

POVEZANOST RADA