Kasabach-merritt syndrome.
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.


CONCLUSIONS
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.
Maguiness
Sheilagh
S
University of Western Ontario, London, Ontario, Canada.
Guenther
Lyn
L
D001132:Arm; D006390:Hemangioendothelioma / Q000151:congenital / Q000473:pathology* / Q000628:therapy*; D006801:Humans; D007223:Infant; D007866:Leg; D013577:Syndrome