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DOI: 10.1055/a-2699-8104
Kaposiform Hemangioendothelioma with Kasabach–Merritt Phenomenon in a Neonate: A Case Report
Autoren
Funding This work was supported by the Science and Technology Project of Jiangxi Provincial Health Commission (grant no. 202310845).
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 × 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.
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.
Keywords
kaposiform hemangioendothelioma - Kasabach–Merritt phenomenon - sirolimus - neonate - thrombocytopenia* These two authors contributed equally to this work and share the first authorship.
Ethical Compliance
This work was approved by the Institutional Ethics Committee of Gannan Medical University, and written informed consent was obtained.
Publikationsverlauf
Eingereicht: 30. Juni 2025
Angenommen: 19. August 2025
Artikel online veröffentlicht:
19. September 2025
© 2025. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution License, permitting unrestricted use, distribution, and reproduction so long as the original work is properly cited. (https://creativecommons.org/licenses/by/4.0/)
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References
- 1 Croteau SE, Liang MG, Kozakewich HP. et al. Kaposiform hemangioendothelioma: atypical features and risks of Kasabach-Merritt phenomenon in 107 referrals. J Pediatr 2013; 162 (01) 142-147
- 2 Ji Y, Chen S, Yang K, Xia C, Li L. Kaposiform hemangioendothelioma: current knowledge and future perspectives. Orphanet J Rare Dis 2020; 15 (01) 39
- 3 Drolet BA, Trenor III CC, Brandão LR. et al. Consensus-derived practice standards plan for complicated Kaposiform hemangioendothelioma. J Pediatr 2013; 163 (01) 285-291
- 4 Zhang X, Yang K, Chen S, Ji Y. Tacrolimus ointment for the treatment of superficial kaposiform hemangioendothelioma and tufted angioma. J Dermatol 2019; 46 (10) 898-901
- 5 Dang N, Ren Y. A case of superficial kaposiform hemangioendothelioma treated with oral propranolol combined with topical sirolimus. Vasc Health Risk Manag 2024; 20: 251-254
- 6 Wang L, Li J, Wu C. et al. Case report: experience of a rare case of rebound of the Kasabach-Merritt phenomenon during sirolimus treatment in kaposiform hemangioendothelioma. Front Pediatr 2022; 10: 949950
- 7 Kalbfell R, Cohen-Cutler S, Grisham E. et al. Infectious complications of vascular anomalies treated with sirolimus: a systematic review. Pediatr Blood Cancer 2024; 71 (01) e30758
- 8 Crary SE. Hemostasis and thrombosis risks and management in vascular anomalies. Hematology (Am Soc Hematol Educ Program) 2024; 2024 (01) 718-723