engleski

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

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.

kaposiform hemangioendothelioma ; Kasabach-Merritt phenomenon ; neonate ; sirolimus

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