Keywords
Kazabach-Meritt syndrome
thrombocytopenia
treatment
How to Cite
Abstract
Introduction. Kaposiform hemangioendothelioma (KHE) is a life-threatening, locally aggressive, and potentially fatal vascular tumor of infancy. KHE is frequently associated with Kasabach-Merritt syndrome (KMS).
Case description. Clinically, KHE typically presents as a firm, irregularly marginated tumor with a reddish-purple hue, nodular surface, with warmth and flaky texture. The disease often manifests with cutaneous manifestations that may mimic infectious processes, thereby complicating diagnosis. Imaging studies are crucial for differentiating KHE from other tumors and assessing disease extent. This article reports a clinical case of KHE with KMS in a newborn infant. Treatment approaches involve various therapeutic combinations, guided by clinical guidelines for infantile hemangiomas and international recommendations for KHE management.
Conclusion. The absence of a standardized treatment protocol for this condition in Russia significantly complicates therapeutic decision-making. Therefore, KHE with KMS should be regarded as a clinically complex, potentially life-threatening entity requiring further investigation.
References
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Copyright (c) 2026 Валерий Петрович Куценко, Владимир Викторович Рязанов , Сергей Сергеевич Багненко , Гульназ Камальдиновна Садыкова, Александра Никитична Оносовская, Дарья Дмитриевна Ковалева