Also known as: KMS, Hemangiomas with thrombocytopenia
What is Kasabach-Merrit Phenomenon?
Kasabach-Merritt syndrome (KMS) is also known as “Hemangiomas with thrombocytopenia.”
It is a combination of hemangioma, thrombocytopenia, and coagulopathy. It is a rare disease of infancy in which the thrombocytopenia (decreased platelet count) results from the hemangioma trapping and activating platelets, which in turn promotes further growth of the vascular tumor. Bleeding problems can ensue and may be life-threatening.
Laboratory testing including blood counts and clotting studies are important, as well as imaging studies and biopsy of the tumor when necessary.
Treatment for Kasabach-Merrit Phenomenon
Management of these patients is complex with several subspecialists involved.
Generally, treatment of the underlying vascular tumor results in resolution of KMS. Surgical resection may provide a cure if it poses no threat to the patient.
Other treatments include embolization and compression bandages to decrease the tumor’s blood supply, corticosteroids, alpha-interferon, and chemotherapy.
Mortality rate is about 30%. Patients need close follow-up with Dermatologists for residual cosmetic lesions.
This page was last updated on: 3/23/2018 1:55:23 PM
From the Newsdesk
In observance of vascular birthmarks awareness month, The International Birthmarks Institute at Nicklaus Children’s Hospital held its first Vascular Birthmarks Conference at the hospital’s main campus on May 5th. The event brought together patients, families and medical professionals representing a range of specialties to present the latest in diagnosis, treatment and research related to birthmarks.
Just a few weeks after Brianna was born, her mother noticed a red growth on her daughter’s upper lip. Her pediatrician referred the family to specialists who diagnosed the growth as an Infantile Hemangioma. On December 7th, Dr. Chad Perlyn of Nickalus Children's Hospital, removed the hemangioma.