Kasabach Merritt syndrome
Kasabach-Merritt Syndrome
Kasabach-Merritt Syndrome (pronunciation: kah-suh-bach merr-it sin-drome) is a rare hematologic disorder characterized by the presence of a vascular tumor and a severe coagulopathy resulting from platelet trapping within the tumor. The syndrome was first described by Haig Haigouni Kasabach and Katharine Krom Merritt in 1940.
Etymology
The syndrome is named after the two pediatricians, Haig Haigouni Kasabach and Katharine Krom Merritt, who first described the condition in 1940.
Symptoms
The primary symptoms of Kasabach-Merritt Syndrome include the presence of a rapidly growing vascular tumor, usually present at birth or appearing in the first few weeks of life, and a severe coagulopathy resulting from platelet trapping within the tumor. Other symptoms may include anemia, thrombocytopenia, and disseminated intravascular coagulation.
Diagnosis
Diagnosis of Kasabach-Merritt Syndrome is typically made based on the presence of a rapidly growing vascular tumor and laboratory findings of a severe coagulopathy. Additional diagnostic tests may include ultrasound, magnetic resonance imaging (MRI), and biopsy of the tumor.
Treatment
Treatment of Kasabach-Merritt Syndrome typically involves addressing the underlying vascular tumor and managing the coagulopathy. This may include surgery, radiation therapy, chemotherapy, and/or immunotherapy. In some cases, blood transfusion may be necessary to manage anemia and thrombocytopenia.
See Also
- Hematologic Disorders
- Vascular Tumors
- Coagulopathy
- Anemia
- Thrombocytopenia
- Disseminated Intravascular Coagulation
External links
- Medical encyclopedia article on Kasabach Merritt syndrome
- Wikipedia's article - Kasabach Merritt syndrome
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