![]() Yellowing of the skin (due to liver irritation)ĭiagnosis of Hemophagocytic Lymphohistiocytosis.Symptoms of hemophagocytic lymphohistiocytosis include: Symptoms depend on where the histiocytes (abnormal cells) accumulate. Initially, HLH appears to be a routine febrile (fever) illness, but it lasts longer and children begin showing symptoms uncommon in typical infections, such as yellowing, pale or bruised skin. This disorder can sometimes be seen in children with arthritis. Cancer and some autoimmune disorders (the immune system attacks body tissues and organs) may also trigger secondary HLH. Secondary HLH is often triggered by viral infections such as Epstein-Barr, cytomegalovirus or herpes. It occurs more often in older children and adults. Secondary hemophagocytic lymphohistiocytosis.If a parent has or had HLH, or a parent knows that HLH occurs in his or her family, it is a good idea to visit a genetic counselor before having a baby. Often, children with these genes are diagnosed at very young ages. Several different genes have been identified for primary HLH. In primary HLH, one or both parents carry an abnormal gene that puts a child at risk for HLH. Primary HLH is caused by an inherited (passed down from parents to children) gene mutation. Primary hemophagocytic lymphohistiocytosis.Doctors at Riley at IU Health do special genetic testing to make the correct diagnosis. They can be difficult to tell apart because symptoms can be similar. There are two types of HLH: primary and secondary. It is important to recognize and begin treatment as soon as possible since the condition can progress very rapidly. If untreated, HLH can become fatal very quickly. About 70 percent of cases occur before the age of 1. HLH is primarily a condition that affects infants and small children. This buildup and cell destruction can lead to organ damage and multi-organ failure. These confused histiocytes tend to accumulate in areas where they should not. HLH causes histiocytes to release chemicals into the blood and ingest other cells, mistaking them for an infection. The result can be damage to organs such as the brain, liver and bone marrow. With HLH, histiocytes become overactive and begin to attack the body’s own tissues as if they are invading organisms. ![]() Normal histiocytes help control infections and remove dead cells from the body. Normal histiocytes are a type of white blood cell found in the blood and in organs such as the lymph nodes, spleen and liver. The abnormal cells in HLH are called histiocytes. ![]()
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