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Potentially life-threatening emergency
Kasabach-Merritt syndrome in Child
Other Resources UpToDate PubMed
Potentially life-threatening emergency

Kasabach-Merritt syndrome in Child

Contributors: Craig N. Burkhart MD, Dean Morrell MD, Lowell A. Goldsmith MD, MPH, Keith Morley MD
Other Resources UpToDate PubMed

Synopsis

Kasabach-Merritt syndrome (KMS), also known as Kasabach-Merritt phenomenon, is characterized by an enlarging vascular lesion, profound thrombocytopenia, consumptive coagulopathy, and often a microangiopathic hemolytic anemia. KMS is associated with vascular tumors, especially kaposiform hemangioendothelioma (KHE) and tufted angioma (TA). Whether KMS complicates true hemangiomas of infancy is controversial, although there have been cases associated with diffuse neonatal hemangiomatosis and visceral hemangiomas.

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.

Codes

ICD10CM:
D69.49 – Other primary thrombocytopenia

SNOMEDCT:
86635005 – Kasabach-Merritt syndrome

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Last Updated:01/17/2022
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Potentially life-threatening emergency
Kasabach-Merritt syndrome in Child
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A medical illustration showing key findings of Kasabach-Merritt syndrome : Abdominal distension, Painful skin lesions, Tumor, Vascular plaque, Ecchymosis, PT prolonged, HR increased, PLT decreased, RBC decreased
Clinical image of Kasabach-Merritt syndrome - imageId=1859794. Click to open in gallery.
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