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: 10/4/2019 11:55:14 AM