Kasabach-Merritt syndrome in Child
Affected infants typically present with a solitary cutaneous lesion either at birth or within the first year of life, although multiple cutaneous or visceral vascular tumors are also encountered in affected infants. KMS is thought to result from platelet trapping within the hemangioma, with subsequent consumption of clotting factors, leading to intralesional bleeding and a rapid enlargement of the vascular lesion. In the midst of the consumptive coagulopathy, easy bruising and even frank bleeding occur.
The rapidly enlarging vascular lesion can compress vital organs and may lead to high-output heart failure. KMS has been associated with a mortality of between 30%-40%, mostly secondary to hemorrhage.
For more information, see OMIM.
D69.49 – Other primary thrombocytopenia
86635005 – Kasabach-Merritt syndrome
- Kaposiform hemangioendothelioma
- Tufted angioma
- Diffuse neonatal hemangiomatosis
- Klippel-Trenaunay syndrome
- Blue rubber bleb nevus syndrome
- Hepatic hemangioma