Combination therapy for pediatric patients with Kasabach-Merritt phenomenon: A single-center retrospective study.

Abstract

This study aimed to in the management of Kasabach-Merritt phenomenon (KMP), a severe thrombocytopenic coagulopathy that occurs in the presence of an enlarging vascular tumor. Here, we retrospectively evaluated 12 patients with KMP in Guangzhou Women and Children's Medical Center, Guangzhou Medical University, from 2017 to 2021. 12 patients, including 7 females and 5 males, were identified. Tumors were located in the leg (n = 4), neck (n = 1), face (n = 3), chest wall (n = 1), back (n = 2), and retroperitoneum (n = 1). A plaque-like lesion with ecchymosis was the most common cutaneous manifestation. All the patients underwent embolization therapy. Nine patients received steroid treatment and 7 patients were administered with sirolimus. The mean duration of treatment was 1.6 months. All the patients reported in this study were alive when discharged. Embolization combined with steroid and sirolimus appears effective in patients with KMP, as well as in those who experienced disease recurrence. However, a long-term follow-up of the children cured of KMP will be necessary to monitor its recurrence and improve the outcome.

• Authors: Li Zhang, Lang Liu, Huanmin Luo, Wenbiao Xu, Huishan Chen, Muxia Yan, Yiqian Wang
• Journal: Medicine (Medicine (Baltimore)) Vol. 101 Issue 34 Pg. e30296 (Aug 26 2022) ISSN: 1536-5964 [Electronic] United States
• PMID: 36042656 (Publication Type: Journal Article)
• Copyright: Copyright © 2022 the Author(s). Published by Wolters Kluwer Health, Inc.

Chemical References

• Sirolimus

Topics

• Child
• Combined Modality Therapy
• Female
• Hemangioendothelioma
• Humans
• Infant
• Kasabach-Merritt Syndrome (drug therapy, pathology)
• Male
• Neoplasm Recurrence, Local
• Retrospective Studies
• Sarcoma, Kaposi
• Sirolimus