Kasabach-Merritt syndrome

Kasabach–Merritt syndrome
Synonyms	Hemangioma-thrombocytopenia syndrome
Classification and external resources
Specialty	hematology
ICD-10	D69.5 (ILDS D69.507)
ICD-9-CM	287.39
OMIM	141000
DiseasesDB	30701
eMedicine	med/1221 ped/1234
Orphanet	2330
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Kasabach–Merritt syndrome (KMS), also known as Hemangioma with thrombocytopenia is a rare disease, usually of infants, in which a vascular tumor leads to decreased platelet counts and sometimes other bleeding problems, which can be life-threatening. It is also known as hemangioma thrombocytopenia syndrome. It is named after Haig Haigouni Kasabach and Katharine Krom Merritt, the two pediatricians who first described the condition in 1940.

KMS is usually caused by a hemangioendothelioma or other vascular tumor, often present at birth. 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, retroperitoneum, 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.

Hemolytic anemia secondary to microangiopathic destruction (physical damage) of the RBCs can be expressed as mild, moderate, or severe.

The diagnostic workup 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.

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