Hemangioma Syndrome

Hemangioma syndrome is also known as hemangioma thrombocytopenia syndrome. In the rare instances that this disease manifests itself, it is most often found in infants. This disease involves the growth of tumors in the blood vessels (hemangioma), which leads to a decrease in blood platelets (thrombocytopenia). Hemangioma syndrome is a life-threatening disease, and people who suffer from extreme cases of it have on average a 70 percent chance of survival.

Identification

• In 1940 Dr. Haig Haigouni Kasabach and Dr. Katharine Krom Meritt first investigated hemangioma syndrome. Their names were attached to the disease in honor of their valuable medical findings, thus it is now also known as Kasabach-Meritt syndrome (KMS). KMS is actually a combination of three conditions: hemangioma, thrombocytopenia, and consumptive coagulopathy. All three must be present in the patient for him to be diagnosed with KMS.

Hemangioma

• Hemangioma is characterized by tumors that develop from the endothelial cells, which are the cells that make up the lining of blood vessels. They represent one type among many possible vascular tumors and are often benign. Hemangioma commonly develops in infants, manifesting during a baby's first few days or weeks and then gradually decreasing in size as the child grows older. The tumors can appear near the surface of the skin, but they can also be present within internal organs. Instead of resolving, these vascular tumors can become malignant and grow very rapidly. This sudden increase in size interferes with the regular circulation of blood and can easily lead to other complications.

Thrombocytopenia

• Platelets or thrombocytes are blood cells that are mostly responsible for blood coagulation. The normal amount of platelets in humans ranges from 150,000 to 450,000 per cubic millimeter of blood. When the number of platelets decreases below this normal range, thrombocytopenia is said to have occurred. Although doctors state that this condition is not necessarily and always the direct result of vascular tumors, hemangiomas that have grown quite large can trap platelets and, thus, decrease the number of these cells in the blood. The usual outward signs of thrombocytopenia are the sudden appearance of bruises on the forearms, nosebleeds and bleeding gums.

Consumptive Coagulopathy

• Another name for consumptive coagulopathy is disseminated intravascular coagulation (DIC). The blood-clotting mechanisms of the circulatory system may react negatively to the presence of vascular tumors. This reaction takes the form of small blood clots building up in the blood vessels. These blood clots in turn eat up the coagulation proteins and platelets (hence the name "consumptive"). With the loss of coagulation proteins and platelets, abnormal bleeding will occur. These blood clots also block the normal flow of blood in vital organs and cause them to malfunction.

Treatment

• Hemangioma syndrome is a rare disease and thus no standard set of guidelines for treatment has yet been devised. Various solutions can be applied, but each has its risks. Thrombocytopenia and consumptive coagulopathy can be handled through platelet transfusions and the use of anticoagulant medication, respectively. The dangers that come with frequent transfusions include fluid overload and heart failure. To treat the vascular tumor itself, its blood supply must be blocked. This can be achieved either through radiology methods or compression bandages. Medications that can be utilized include corticosteroids,which are a type of steroid hormone, and alpha-interferon, which are proteins that are part of the immune system. Certain medicines used in chemotherapy can also be used.