Kasabach-Merritt syndrome pathophysiology
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Pathophysiology
KMS is usually caused by a hemangioendothelioma or other vascular tumor, often present at birth.[1][2] Although these tumors are relatively common, it is rare for them to cause KMS.
When these tumors are large or are growing rapidly, sometimes they can trap platelets, causing severe thrombocytopenia. The combination of vascular tumor and consumptive thrombocytopenia defines KMS. Tumors can be found in the trunk, upper and lower extremities, retroperioneum, and in the cervical and facial areas.
This consumptive coagulopathy also uses up clotting factors, such as fibrinogen which may worsen bleeding. The coagulopathy can progress to disseminated intravascular coagulation and even death.
References
- ↑ Enjolras O, Wassef M, Mazoyer E, Frieden I, Rieu P, Drouet L, Taïeb A, Stalder J, Escande J (1997). "Infants with Kasabach-Merritt syndrome do not have "true" hemangiomas". J Pediatr. 130 (4): 631–40. PMID 9108863.
- ↑ el-Dessouky M, Azmy A, Raine P, Young D (1988). "Kasabach-Merritt syndrome". J Pediatr Surg. 23 (2): 109–11. PMID 3278084.