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eISSN: 2300-8660
ISSN: 0031-3939
Pediatria Polska - Polish Journal of Paediatrics
Bieżący numer Archiwum Artykuły zaakceptowane O czasopiśmie Rada naukowa Bazy indeksacyjne Kontakt Zasady publikacji prac Standardy etyczne i procedury
Panel Redakcyjny
Zgłaszanie i recenzowanie prac online
SCImago Journal & Country Rank
1/2020
vol. 95
 
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Opis przypadku

Kasabach-Merritt syndrome in an infant with cavernous haemangioma

Halyna Pavlyshyn
1
,
Nataliia Luchyshyn
1

  1. Department of Paediatrics #2, Ivan Horbachevsky Ternopil National Medical University, Ternopil, Ukraine
Pediatr Pol 2020; 95 (1): 48–51
Data publikacji online: 2020/03/31
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Kasabach-Merritt syndrome (KMS) is a potentially life-threatening consumptive coagulopathy associated with vascular malformations, whereas haemangiomas typically present in infancy, with rapid growth, thoracic location, and complicated clinical course demanding chemotherapy and radiotherapy have rarely been reported. We describe a case of a female infant with cavernous haemangioma of the right chest wall spreading to the pleural space and diaphragm, and complicated by KMS. Our patient presented an inadequate response to treatment with systemic corticosteroids, propranolol, and vincristine. Partial tumour regression and diminish of trapping of platelets in the tumour were achieved after radiotherapy only. The purpose of this report is to increase awareness about KMS, to illustrate the treatment challenges and to provide a basis for clinical decision-making in this rare, life-threatening condition.