Kaposiform hemangioendothelioma (KHE) is a benign tumor of infancy that is often associated with consumption of platelets (the blood cells responsible for blood clotting).When kaposiform hemangioendothelioma destroys platelets, the condition is often referred to as Kasabach-Merritt Syndrome.

KHE and Kasabach-Merritt Syndrome usually appear in the first three months of life, initially as a raised area without or with red or purple discoloration of the skin. Typically, it enlarges suddenly and becomes dark purple in color, due to bleeding into the soft tissues. At the same time, if the blood platelet count is low, the infant will show multiple tiny red spots on the skin (petechiae).

KHE is usually diagnosed by clinical examination when an infant has the typical, rapidly enlarging, purple colored soft tissue mass with an extremely low platelet count. MRI is also useful for making or confirming the diagnosis, as the tumor has fairly typical findings. However, since this is an uncommon tumor, not all physicians are familiar with the typical findings. In some cases, a needle biopsy or surgical biopsy is necessary to make the diagnosis.

KHE and Kasabach-Merritt Syndrome are treated with drugs that interfere with angiogenesis (formation of new blood vessels). Typically, steroids such as Prednisolone are given by mouth. If these are ineffective, then I.V. treatment, usually with Vincristine is tried. In resistant cases, multidrug chemotherapy is sometimes used. Embolization and sclerotherapy is effective in some patients, helping to shrink the tumor and restore a normal platelet count. Platelet transfusions should only be given when the infant is actually bleeding, as they can stimulate growth of the tumor.

Approximately, 50% of babies with kaposiform hemangioendothelioma have a good response to drug treatment. In the other 50%, some will require embolization or surgical removal of the tumor. Infants who do not respond to treatment have a risk of death due to hemorrhage. KHE often does not disappear completely. An area of skin discoloration may persist and in rare cases, the tumor can recur later in life.