Retroperitoneal kaposiform hemangioendothelioma with kasabach-merritt phenomenon in children: A case report and review of the literature

Article Pharmacology & Pharmacy

Effective low-dose sirolimus regimen for kaposiform haemangioendothelioma with Kasabach-Merritt phenomenon in young infants

Veroniek E. M. Harbers et al.

Summary: Low-dose sirolimus treatment is safe and effective for infants with kaposiform haemangioendothelioma and Kasabach-Merritt phenomenon, leading to complete hematological response and resolution of the disease.

BRITISH JOURNAL OF CLINICAL PHARMACOLOGY (2022)

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Article Hematology

Sirolimus plus prednisolone vs sirolimus monotherapy for kaposiform hemangioendothelioma: a randomized clinical trial

Yi Ji et al.

Summary: This study compared the efficacy and safety of sirolimus plus prednisolone vs sirolimus monotherapy for treating KHE with KMP. The combination treatment showed improvements in terms of durable platelet responses, median platelet counts, fibrinogen stabilization, and lesion responses compared to sirolimus monotherapy. Patients receiving combination therapy had fewer blood transfusions and a lower incidence of disease sequelae. The study suggests that sirolimus plus prednisolone is a valid treatment option for KHE with KMP.

BLOOD (2022)

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Editorial Material Medicine, General & Internal

Large abdominal purpura of neonatal retroperitoneal kaposiform hemangioendothelioma

Ryo Takemura et al.

Summary: Large abdominal purpuras may be caused by retroperitoneal kaposiform hemangioendothelioma with consumptive coagulopathy. Clinicians should perform serial ultrasonography studies to detect the sings of tumor until the final diagnosis is confirmed.

CLINICAL CASE REPORTS (2021)

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Article Oncology

Additive value of transarterial embolization to systemic sirolimus treatment in kaposiform hemangioendothelioma

Richard Brill et al.

Summary: A study suggests that adjunct transarterial embolization may lead to a quicker resolution of thrombocytopenia in children with KHE and KMP compared to systemic sirolimus alone; however, tumor response and rebound rates were similar between the two treatment groups.

INTERNATIONAL JOURNAL OF CANCER (2021)

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Article Pediatrics

Kaposiform haemangioendothelioma: magnetic resonance imaging features in 64 cases

Suhua Peng et al.

Summary: Kaposiform haemangioendothelioma (KHE) is a rare and locally aggressive disorder with specific clinical and imaging features, including visible cutaneous lesions, destructive changes in adjacent bone, severe thrombocytopenia, and consumptive coagulopathy, which can aid in the diagnosis of this condition.

BMC PEDIATRICS (2021)

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Review Genetics & Heredity