Definition:
Hemangio thrombocytopenia syndrome or HTS is a rare disease affecting infants characterized by a vascular tumor which causes low platelet count as well as bleeding problems. The condition is also known as Kasabach-Merritt Syndrome.
Diagnosis:
Signs and symptoms of the condition direct the diagnostic workup. This usually includes clotting studies, blood counts, laboratory tests, imaging tests (CT scan, MRI, ultrasound, angiography, and in rare cases, nuclear medicine scans), and biopsy of the tumor.
Treatment:
There is no specific treatment for HTS, although medication and management will depend on the location of platelets, number of lesions, and blood platelet levels. Individuals have options for supportive care and definitive treatment.
Surgery provides a good option for curing the HTS, as well as other techniques that include embolization, compression bandages, radiation therapy, and certain medications (corticosteroids, chemotherapy, alpha-interferon).
Symptoms and Signs:
Symptoms include brownish-red and hard lesions on the skin, usually found on the trunk area and may be painful; internal hemangiomas located in the spleen, liver, or gastrointestinal tract and may possibly result in internal bleeding; and prolonged bleeding.
Causes:
The cause of HTS is unknown; however it is suspected it occurs only in certain types of hemangiomas: tufted angiomas and Kaposi hemangioendotheliomas.
:
hemangiomathrombocytopeniasyndrome
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