Kaposiform lymphangiomatosis

Kaposiform lymphangiomatosis (KLA) is a very rare disorder of the lymphatic system, which is the network of vessels and tissues that helps your body fight infection and maintain fluid balance. In KLA, abnormal lymphatic tissue grows in an uncontrolled way and spreads through multiple organs, most commonly the lungs, bones, spleen, and the mediastinum (the area between the lungs). Unlike some other lymphatic malformations that stay in one spot, KLA tends to involve many parts of the body at once. The abnormal tissue has a distinctive appearance under the microscope, with spindle-shaped cells that resemble those seen in another condition called kaposiform hemangioendothelioma, which is how it got its name. Key symptoms include fluid collections around the lungs (pleural effusions) and around the heart (pericardial effusions), bleeding problems, bone pain or bone destruction, abdominal swelling, and breathing difficulties. Many patients develop a serious bleeding complication called consumptive coagulopathy, where the body uses up clotting factors faster than it can make them. This can lead to dangerous bleeding episodes. Treatment for KLA is challenging because there is no cure. The drug sirolimus (also known as rapamycin), which targets the mTOR pathway, has become the most widely used treatment and can help slow disease progression and reduce symptoms in many patients. Other treatments may include drainage of fluid collections, supportive care for bleeding problems, and sometimes chemotherapy-like drugs such as vincristine. Despite treatment, KLA can be life-threatening, especially when it involves the lungs or causes severe bleeding complications.

Bring these to your next appointment

• Q1.Which organs are affected in my case, and how severe is the involvement?,Is sirolimus the right treatment for me, and what side effects should I watch for?,How often will I need blood tests and imaging to monitor the disease?,What are the warning signs that I should go to the emergency room?,Are there any clinical trials or new treatments available for KLA?,Should I be seen at a specialized vascular anomalies center?,What activity restrictions should I follow to reduce bleeding risk?

Common questions about Kaposiform lymphangiomatosis

Related conditions

Other rare diseases commonly researched alongside Kaposiform lymphangiomatosis.

• Gorham-Stout disease→
• Kaposiform hemangioendothelioma→