• Jun Cheng, Yun Zou, Ronghua Fu, Pingliang Jin, Mengyu Huang, Zhiping Wu, Hanxiang Bai, Xiangqun Huang, and Hua Yuan.
• Department of Plastic Surgery, Jiangxi Provincial Children's Hospital, Nanchang, China.
• Medicine (Baltimore). 2024 Apr 5; 103 (14): e37706e37706.

RationaleKaposiform 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.Patient ConcernsA 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.DiagnosisKaposiform hemangioendothelioma combined with Kasabach-Merritt phenomenon.InterventionsThe 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.OutcomesThe tumor basically disappeared on examination and the ultrasound showed a subcutaneous hyperechoic mass with normal blood flow.LessonsSirolimus combined with glucocorticoids is effective in controlling Kasabach-Merritt phenomenon and pathologic biopsy is important for definitive diagnosis.Copyright © 2024 the Author(s). Published by Wolters Kluwer Health, Inc.

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