Familial Hemophagocytic Lymphohistiocytosis – Lymphatic Diseases

The lymphatic system is a network of tissues and organs. It is made up of

• Lymph – a fluid that contains white blood cells that defend against germs
• Lymph vessels – vessels that carry lymph throughout your body. They are different from blood vessels.
• Lymph nodes – glands found throughout the lymph vessels. Along with your spleen, these nodes are where white blood cells fight infection.

Your bone marrow and thymus produce the cells in lymph. They are part of the system, too.

The lymphatic system clears away infection and keeps your body fluids in balance. If it’s not working properly, fluid builds in your tissues and causes swelling, called lymphedema. Other lymphatic system problems can include infections, blockage, and cancer.

• Cancer and lymph nodes (Medical Encyclopedia)
• Cystic hygroma (Medical Encyclopedia)
• Groin lump (Medical Encyclopedia)
• Lymph node biopsy (Medical Encyclopedia)
• Lymph system (Medical Encyclopedia)
• Lymphadenitis (Medical Encyclopedia)
• Lymphangitis (Medical Encyclopedia)
• Lymphofollicular hyperplasia (Medical Encyclopedia)
• Neck lump (Medical Encyclopedia)
• Swollen lymph nodes (Medical Encyclopedia)

Familial hemophagocytic lymphohistiocytosis is a disorder in which the immune system produces too many activated immune cells (lymphocytes) called T cells, natural killer cells, B cells, and macrophages (histiocytes). Excessive amounts of immune system proteins called cytokines are also produced. This overactivation of the immune system causes fever and damages the liver and spleen, resulting in enlargement of these organs.Familial hemophagocytic lymphohistiocytosis also destroys blood-producing cells in the bone marrow, a process called hemophagocytosis. As a result, affected individuals have low numbers of red blood cells (anemia) and a reduction in the number of platelets, which are involved in clotting. A reduction in platelets may cause easy bruising and abnormal bleeding.The brain may also be affected in familial hemophagocytic lymphohistiocytosis. As a result, affected individuals may experience irritability, delayed closure of the bones of the skull in infants, neck stiffness, abnormal muscle tone, impaired muscle coordination, paralysis, blindness, seizures, and coma. In addition to neurological problems, familial hemophagocytic lymphohistiocytosis can cause abnormalities of the heart, kidneys, and other organs and tissues. Affected individuals also have an increased risk of developing cancers of blood-forming cells (leukemia and lymphoma).Signs and symptoms of familial hemophagocytic lymphohistiocytosis usually become apparent during infancy, although occasionally they appear later in life. They usually occur when the immune system launches an exaggerated response to an infection, but may also occur in the absence of infection. Without treatment, most people with familial hemophagocytic lymphohistiocytosis survive only a few months.