Kaposiform Hemangioendothelioma Symptoms & Causes

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Symptoms & causes of kaposiform hemangioendothelioma in children

What are the symptoms of KHE?

The most common symptom of KHE is a growing lesion on the skin. This lesion:

    • is usually a deep reddish-purple and has poorly defined edges
    • is firm and warm to the touch
    • often has shiny and tense skin
    • may be accompanied by a bruise-like discoloration nearby, or tiny red or purple
    spots (petechiae) anywhere on the body
    • sometimes swells and becomes painful, or causes pain with movement or reduced
    range of motion
The exact symptoms of KHE depend on where the lesion appears. About 10 percent of children with KHE have no skin lesion, and some lesions penetrate deeper than the skin into other tissues.

Children with KHE also often have low platelet counts (especially if they develop the Kasabach-Merritt phenomenon; see below).

Is a KHE lesion painful?

Sometimes yes, but not in all cases. As the tumor gets bigger and more aggressive, a lesion can swell and may be very painful. This may also occur with infection, trauma to the lesion or after platelet transfusion.

Are there complications of KHE?

Most patients have few complications and may not need medical therapy. Some patients with KHE, however, develop what is called Kasabach-Merritt phenomenon (KMP), in which their tumor traps platelets and keeps them from circulating through the bloodstream. Because platelets help prevent bleeding, patients with KMP have a higher risk of bleeding.
KMP may develop in patients whose tumors are larger, invade into deeper tissues or if there are sites of infection or inflammation nearby. Some patients who develop KMP also lack other blood proteins that help control bleeding, such as fibrinogen.

Does KHE always lead to Kasabach-Merritt phenomenon?

Not always, although nearly 75 percent of cases referred to the Vascular Anomalies Center at Boston Children’s have or develop KMP. However, as the VAC tends to see more severe cases, the percentage of KHE patients with KMP seen at the VAC may be greater than that among all KHE patients.

In very rare situations, KHE involves only bones and superficial tissues (skin and tissue just beneath the skin); these appear to have low risk of Kasabach-Merritt phenomenon.

What causes kaposiform hemangioendothelioma?

Researchers are still searching for the cause of KHE. It is not inherited, and while it typically occurs in infancy, KHE can arise prenatally or in adults after trauma.

Kaposiform hemangioendothelioma: Reviewed by Cameron C. Trenor III, MD, © Boston Children's Hospital, 2016

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