Kasabach-merritt syndrome

J Cutan Med Surg. 2002 Jul-Aug;6(4):335-9. doi: 10.1177/120347540200600405. Epub 2002 Apr 15.


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.

Publication types

  • Review

MeSH terms

  • Arm
  • Hemangioendothelioma / congenital
  • Hemangioendothelioma / pathology*
  • Hemangioendothelioma / therapy*
  • Humans
  • Infant
  • Leg
  • Syndrome