Background: Kasabach–Merritt syndrome (KMS) is a consumptive coagulopathy associated with the presence of a large vascular lesion. It is often a frustrating condition to treat and it carries a high mortality rate. There are currently no known treatment guidelines. Kasabach–Merritt syndrome is associated with kaposiform hemangioendothelioma (KHE) and tufted angioma (TA); these lesions, when associated with KMS, are locally invasive, aggressive vascular tumors. Treatment options include supportive care, local therapies, and drug and surgical management. In most recent case reports, a multimodal approach to therapy is taken. Objective and Conclusion: The objective of this article is to provide a comprehensive review of KMS and give an up-to-date summary of treatment options. The clinical presentation, laboratory findings, vascular pathology, and pathophysiology will also be discussed.
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Maguiness, S., Guenther, L. Kasabach–Merritt Syndrome . JCMS 6, 335–339 (2002). https://doi.org/10.1007/s10227-001-0055-4
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DOI: https://doi.org/10.1007/s10227-001-0055-4