Kasabach-Merritt Syndrome
- PMID: 30085595
- Bookshelf ID: NBK519053
Kasabach-Merritt Syndrome
Excerpt
Kasabach-Merritt phenomenon (KMP), first described in 1940, is a rare but life-threatening coagulopathy of infancy which presents with thrombocytopenia, microangiopathic hemolytic anemia, and consumptive coagulopathy in the setting of a rapidly enlarging vascular tumor. It is exclusively associated with the vascular tumors kaposiform hemangioendothelioma (KHE) and tufted angioma (TA), which exist along the same neoplastic spectrum. Treatment includes supportive therapy and management of the underlying tumor.
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References
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- Ji Y, Chen S, Yang K, Xia C, Peng S. Development of Kasabach-Merritt phenomenon following vaccination: More than a coincidence? J Dermatol. 2018 Oct;45(10):1203-1206. - PubMed
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- Schmid I, Klenk AK, Sparber-Sauer M, Koscielniak E, Maxwell R, Häberle B. Kaposiform hemangioendothelioma in children: a benign vascular tumor with multiple treatment options. World J Pediatr. 2018 Aug;14(4):322-329. - PubMed
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