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Potentially life-threatening emergency
Kasabach-Merritt syndrome - Skin
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Potentially life-threatening emergency

Kasabach-Merritt syndrome - Skin

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Contributors: Craig N. Burkhart MD, Dean Morrell MD, Lowell A. Goldsmith MD, MPH, Nancy Esterly 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, though 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, though 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.

Codes

ICD10CM:
D69.49 – Other primary thrombocytopenia

SNOMEDCT:
86635005 – Kasabach-Merritt syndrome

Look For

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

Vascular lesions associated with KMS:
Other conditions to consider:

Best Tests

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Management Pearls

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Therapy

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References

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Last Updated: 03/29/2017
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Potentially life-threatening emergency
Kasabach-Merritt syndrome - Skin
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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
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