Blood: Sirolimus + Prednisolone vs Sirolimus Monotherapy for Kaposi-like Hemangioendothelioma

Kaposi-like hemangioendothelioma (KHE) is a rare, aggressive vascular tumor that occurs mainly in infants and children and resembles Kaposi's sarcoma
.

The Kasabach-Merritt phenomenon (KMP) of Kaposi-like hemangioendothelioma, characterized by life-threatening thrombocytopenia and consumptive coagulopathy, occurs in approximately 70% of patients with Kaposi-like hemangioendothelioma

The Kasabach-Merritt phenomenon (KMP) of Kaposi-like hemangioendothelioma, characterized by life-threatening thrombocytopenia and consumptive coagulopathy, occurs in approximately 70% of patients with Kaposi-like hemangioendothelioma

This study is a multicenter, randomized, controlled, open-label clinical trial to compare sirolimus plus prednisolone versus sirolimus monotherapy for carboxylate with Kasabach-Merritt phenomenon Efficacy and safety of hemangioendothelioma
.

Patients were randomly assigned to receive either sirolimus plus a short course of prednisolone or sirolimus monotherapy for at least 12 months
.

The primary endpoint was sustained platelet remission rate (platelet count >100x109/L) at week 4

Changes in platelet counts in the two groups

Changes in platelet counts in the two groups

A total of 73 patients (37 in the combination group and 36 in the single-agent group) were recruited
.

At week 4, 35 (94.

At week 4, 35 (94.

The occurrence of side effects in the two groups

The occurrence of side effects in the two groups

Patients who received the combination therapy had fewer blood transfusions and had lower rates of complications
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There was no significant difference in the incidence of grade 3-4 adverse events during the study treatment period between the two groups

Fewer transfusions and lower complication rates in patients receiving combination therapy

Lesions of a child before and after combined therapy

Lesions of a child before and after combined therapy

In conclusion, the results of this study show that the combination of sirolimus and prednisolone in the treatment of patients with Kasabach-Merritt phenomenon with Kaposi-like hemangioendothelioma is expected to be effective and safe, and this combination method may become such a Effective treatment options for patients

The efficacy of sirolimus combined with prednisolone in the treatment of patients with Kaposi-like hemangioendothelioma with Kasabach-Merritt phenomenon is promising and safe

 

Original source:

Original source:

Yi Ji, et al.

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