Kaposiform Lymphangiomatosis (KLA) is considered different from GLA, based on the behaviour of cells and molecules in the lesions. Patients with KLA often present with a growing mass of abnormal lymphatic tissue in the chest and abdomen. Bleeding and breathing problems are common. Patients with KLA may have a worse prognosis than GLA patients.

KLA is associated with a somatic NRAS mutation.

Why is it more dangerous than GLA?

KLA can be distinguished from GLA by the associated clotting abnormalities and presence of spindle-shaped cells under a microscope.

Who has KLA?

KLA can occur at any age, but the incidence is highest in children and teenagers. Signs and symptoms are typically present before the age of 20. The condition is often under-recognized in adults. It strikes males and females of all races and exhibits no inheritance pattern. The medical literature contains case reports from every continent.

After Michio Ozeki, Toshiyuki Fukoa, 2019.