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Kasabach-Merritt syndrome (KMS) is a rare disease, usually of infants, in which a vascular tumor leads to decreased platelet counts and sometimes other bleeding problems,[1] which can be life-threatening.[2] It is also known as hemangioma thrombocytopenia syndrome. It carries the names of Dr Haig Haigouni Kasabach and Dr Katharine Krom Merritt, the two pediatricians who first described the condition in 1940.[3][4]
KMS is usually caused by a hemangioendothelioma or other vascular tumor, often present at birth.[5][6] 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.[1]
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.[1]
The diagnostic workup[7] is directed by the presenting signs and symptoms, and can involve:
Patients uniformly show severe thrombocytopenia, low fibrinogen levels, high fibrin degradation products (due to fibrinolysis), and microangiopathic hemolysis.[1]
Management of KMS, particularly in severe cases, can be complex and require the joint effort of multiple subspecialists. This is a rare disease with no consensus treatment guidelines or large randomized controlled trials to guide therapy.
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