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Kasabach–Meritt syndrome, also known as hemangioma with thrombocytopenia.
A rare disease, usually of infants, in which a vascular tumor leads to decreased platelet counts and sometimes other bleeding problems.
Can be life-threatening.
Kasabach–M2242itt syndrome is usually caused by a hemangioendothelioma or other vascular tumor, often present at birth.
These tumors are relatively common, but only rarely cause Kasabach–Merritt syndrome.
These tumors are large or are growing rapidly, and can trap platelets, causing severe thrombocytopenia.
Tumors can be found in the trunk, upper and lower extremities, retroperitoneum, and in the cervical and facial areas.
A consumptive coagulopathy also occurs using up clotting factors, such as fibrinogen which may worsen bleeding.
The consumptive coagulopathy can progress to disseminated intravascular coagulation and even death.
Microangiopathic hemolytic anemia destruction can be mild, moderate, or severe.
The diagnostic workup includes: blood counts, clotting studies, ultrasound, CT scan, MRI, sometimes angiography, and rarely nuclear medicine scans.
Biopsy is contraindicated due to risk of bleeding.
Patients uniformly show severe thrombocytopenia, low fibrinogen levels, high fibrin degradation products, and microangiopathic hemolysis.
No consensus treatment guidelines or large randomized controlled trials to guide therapy.
Patients are at risk of bleeding complications including intracranial hemorrhage.
The thrombocytopenia and coagulopathy are managed with platelet transfusions and fresh frozen plasma.
Treatment of the underlying vascular tumor results in resolution of Kasabach–Merritt syndrome.
If complete surgical resection is feasible, it provides a good opportunity for cure.
It can be dangerous to operate on a vascular tumor.
If surgery is not possible:
embolization can limit the tumor’s blood supply
external compression bandages can have similar effects
corticosteroids
alpha-interferon
chemotherapy
It has a mortality rate of about 30%.