Kasabach-Merritt syndrome

Kasabach-Merritt syndrome (KMS), also known as hemangioma-thrombocytopenia syndrome, is a rare disorder characterized by profound thrombocytopenia, microangiopathic hemolytic anemia, and subsequent consumptive coagulopathy in association with vascular tumors, particularly kaposiform hemangioendothelioma or tufted angioma.

Vascular neoplasm

A benign or malignant neoplasm (tumour) originating in the vascular system.


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PMID (PMCID)
24738105
 FEMALE Infant, Newborn
Kaposiform hemangioendothelioma with Kasabach-Merritt syndrome mistaken for child abuse in a newborn.
Cyrulnik AA, Dawkins MC, Smalberger GJ, Young S, Mann RE, Jacobson MI, Friedman AJ.
Cutis. 2014;93(3):E17-20.
Biopsy demonstrated a vascular neoplasm consistent with kaposiform hemangioendothelioma (KHE), and laboratory evaluation revealed thrombocytopenia, low fibrinogen levels, and elevated D-dimer levels, confirming a diagnosis of Kasabach-Merritt syndrome (KMS).
15163230
 FEMALE
Kaposiform hemangioendothelioma associated with nonimmune fetal hydrops.
Martinez AE, Robinson MJ, Alexis JB.
Arch Pathol Lab Med. 2004;128(6):678-81.
This rare vascular neoplasm usually presents as a localized violaceous skin lesion in infants and behaves in a benign fashion; however, kaposiform hemangioendothelioma may present as an invasive neoplasm of the chest or abdominal cavity, where it can lead to the Kasabach-Merritt syndrome, which consists of thrombocytopenia, consumptive coagulopathy, and microangiopathic anemia in association with a vascular anomaly.