Kaposiform Hemangioendothelioma (KHE) | Symptoms & Causes

What are the symptoms of KHE?

The exact symptoms of KHE depend on where the lesion appears. 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.

Most children 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 children who develop KMP also lack other blood proteins that help control bleeding, such as fibrinogen.

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.