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Kaposiform Hemangioendothelioma with Kasabach–Merritt Phenomenon in a Neonate: A Case Report

Year: 2025

Type: article

Abstract: Kaposiform hemangioendothelioma (KHE) is a rare, aggressive vascular tumor frequently complicated by Kasabach–Merritt phenomenon (KMP), a life-threatening consumptive coagulopathy. Neonatal KHE-KMP management requires urgent intervention but is complicated by immunosuppression risks, especially in Bacillus Calmette–Guérin (BCG)-vaccinated infants. A full-term male neonate with prenatal right upper limb thickening presented postnatally with a violaceous, firm mass. Laboratory findings confirmed KMP. Due to recent BCG vaccination, sirolimus was initially withheld. First-line therapies failed, prompting sirolimus initiation on day 3, supplemented by fibrinogen transfusions. Platelets normalized by day 13 (283 × 109/L) with marked tumor regression. Transient fever/diarrhea resolved with supportive care. At discharge (day 27), platelets stabilized (183 × 109/L). Three-month follow-up showed sustained platelet recovery (268–532 × 109/L), near-complete tumor resolution, and age-appropriate development. Prophylactic trimethoprim-sulfamethoxazole prevented infections. Sirolimus is a critical salvage therapy for refractory neonatal KHE-KMP, even in BCG-vaccinated infants. Timely initiation reverses life-threatening coagulopathy and achieves favorable outcomes, necessitating multidisciplinary monitoring to balance immunosuppression risks.

Source: European Journal of Pediatric Surgery Reports

DOI: 10.1055/a-2699-8104

Authors: Yuyang Zheng, Rongjie Wu, Qilin Chen, Shiyu Xiong, Jialu Yun, Wei Peng

Institutions: Gannan Medical University, First Affiliated Hospital of Gannan Medical University

Language: English

Topic: Vascular Malformations and Hemangiomas

Subfield: Surgery

Field: Medicine

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: gold

Estimated APC paid: $1,700