{"title":"Kaposiform Hemangioendothelioma with Kasabach-Merritt Phenomenon in a Neonate: A Case Report.","authors":"Yuyang Zheng, Rongjie Wu, Qilin Chen, Shiyu Xiong, Jialu Yun, Wei Peng","doi":"10.1055/a-2699-8104","DOIUrl":null,"url":null,"abstract":"<p><strong>Background: </strong>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.</p><p><strong>Methods: </strong>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.</p><p><strong>Results: </strong>Platelets normalized by day 13 (283 × 10 <sup>9</sup> /L) with marked tumor regression. Transient fever/diarrhea resolved with supportive care. At discharge (day 27), platelets stabilized (183 × 10 <sup>9</sup> /L). Three-month follow-up showed sustained platelet recovery (268-532 × 10 <sup>9</sup> /L), near-complete tumor resolution, and age-appropriate development. Prophylactic trimethoprim-sulfamethoxazole prevented infections.</p><p><strong>Conclusion: </strong>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.</p>","PeriodicalId":43204,"journal":{"name":"European Journal of Pediatric Surgery Reports","volume":"13 1","pages":"e155-e158"},"PeriodicalIF":0.7000,"publicationDate":"2025-09-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12449040/pdf/","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"European Journal of Pediatric Surgery Reports","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1055/a-2699-8104","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2025/1/1 0:00:00","PubModel":"eCollection","JCR":"Q4","JCRName":"SURGERY","Score":null,"Total":0}
引用次数: 0
Abstract
Background: 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.
Methods: 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.
Results: Platelets normalized by day 13 (283 × 10 9 /L) with marked tumor regression. Transient fever/diarrhea resolved with supportive care. At discharge (day 27), platelets stabilized (183 × 10 9 /L). Three-month follow-up showed sustained platelet recovery (268-532 × 10 9 /L), near-complete tumor resolution, and age-appropriate development. Prophylactic trimethoprim-sulfamethoxazole prevented infections.
Conclusion: 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.
求助内容：
应助结果提醒方式：
京公网安备 11010802042870号
