Kasabach–Merritt syndrome

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Kasabach–Merritt syndrome (pronunciation: kah-suh-bak merr-it sin-drome) is a rare hematologic disorder that typically presents in infancy. The syndrome is characterized by a rapidly growing vascular tumor, thrombocytopenia, and coagulopathy.

Etymology

The syndrome is named after the American pediatricians Katharine Krom Merritt and Haig Haigouni Kasabach who first described the condition in 1940.

Symptoms

The primary symptoms of Kasabach–Merritt syndrome include a rapidly enlarging hemangioma, severe thrombocytopenia, and consumptive coagulopathy. Other symptoms may include anemia, edema, and pain.

Diagnosis

Diagnosis of Kasabach–Merritt syndrome is typically made based on clinical presentation and laboratory findings. Imaging studies such as ultrasound, MRI, or CT scan may be used to further evaluate the extent of the vascular tumor.

Treatment

Treatment of Kasabach–Merritt syndrome often involves a multidisciplinary approach and may include medical therapy, surgical intervention, and supportive care. Medical therapies may include corticosteroids, vincristine, and sirolimus. In severe cases, surgical intervention may be necessary.

Prognosis

The prognosis of Kasabach–Merritt syndrome varies and is dependent on the size and location of the vascular tumor, as well as the severity of the coagulopathy and thrombocytopenia.

See also

External links

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