%0 Case Reports
%T Sirolimus combined with glucocorticoids in the treatment of Kasabach-Merritt phenomenon in a neonate: A case report.
%A Cheng J
%A Zou Y
%A Fu R
%A Jin P
%A Huang M
%A Wu Z
%A Bai H
%A Huang X
%A Yuan H
%J Medicine (Baltimore)
%V 103
%N 14
%D 2024 Apr 5
%M 38579031
%F 1.817
%R 10.1097/MD.0000000000037706
%X <B>BACKGROUND: </B>Kaposiform hemangioendothelioma is an aggressive vascular tumor that is often associated with life-threatening coagulopathies and Kasabach-Merritt phenomenon. Pathologic biopsies can provide a good basis for diagnosis and treatment. Therapy with srolimus combined with glucocorticoids may offer patients a favorable prognosis.<BR><B>METHODS: </B>A large purplish-red mass on the knee of a child with extremely progressive thrombocytopenia and refractory coagulation abnormalities. Conventional doses of glucocorticoids alone failed to improve coagulation abnormalities and the child developed large cutaneous petechiae and scalp hematomas.<BR><B>METHODS: </B>Kaposiform hemangioendothelioma combined with Kasabach-Merritt phenomenon.<BR><B>METHODS: </B>The patient received prednisolone 2.0 mg/kg*d for 4 days. Blood products were transfused to ensure vital signs and to complete the pathologic biopsy. Sirolimus combined with prednisolone was given after clarifying the diagnosis of Kaposiform hemangioendothelioma.<BR><B>RESULTS: </B>The tumor basically disappeared on examination and the ultrasound showed a subcutaneous hyperechoic mass with normal blood flow.<BR><B>CONCLUSIONS: </B>Sirolimus combined with glucocorticoids is effective in controlling Kasabach-Merritt phenomenon and pathologic biopsy is important for definitive diagnosis.