Symptoms
Symptoms of Kasabach-Merritt syndrome include:
- A large reddish-brown mass (tumor) consisting of abnormal blood vessels (hemangioma) develops, most commonly on the skin. This may grow rapidly and spread over a large area of the body.
- The mass may become painful and the affected skin may become ulcerated.
- If the hemangioma is inside the body, bruising may be seen on the skin near it. It can be life-threatening if the hemangioma spreads to vital organs in the body.
- The lesions causes low levels of platelets in the blood (thrombocytopenia). This leads to abnormal bleeding (such as from the gums) and frequent bruising. Thrombocytopenia can be life-threatening.
Diagnosis
Once the hemangioma has been found, a sample of its tissue (biopsy) can help confirm the diagnosis of Kasabach-Merritt syndrome. A complete blood count (CBC) is done to check the level of platelets in the blood.
Treatment
If an individual with Kasabach-Merritt syndrome has a low level of platelets in the blood, he or she will often be admitted to the hospital for treatment, and may require platelet transfusion. The hemangioma may be treated with different medications. Corticosteroids such as prednisone, antitumor drugs such as interferon alfa or vincristine, or other drugs may be used. Radiation therapy had been used in the past but is no longer favored due to the risk of the radiation causing cancer later on in life.
Surgery may be done if the hemangioma is small enough to be taken out, but usually in Kasabach-Merritt syndrome it is too large to be removed. Sometimes a vascular surgeon may be able to stop the blood flow in the blood vessels leading to the hemangioma through arterial embolization.
In many individuals, the tumor shrinks and resolves with treatment. In others, the clotting problem may resolve but the tumor may remain.
Information for this article was taken from:
- Cincinnati Children’s Hospital Medical Center. Kasabach-Merritt Phenomenon.
- Krafchik, B. R., & Hendricks, L. K. (2004). Kasabach-Merritt syndrome. eMedicine, accessed at http://www.emedicine.com/med/topic1221.htm
