Hemophagocytic lymphohistiocytosis, also known as HLH, is a rare immune disorder that causes the immune system to malfunction and attack the body. HLH is caused by white blood cells building up in organs, causing the organ to expand to an abnormal size. If a person goes untreated for 2 months after getting the disease, it is highly likely that the patient will die from it. HLH can cause inflammation of the brain, which can lead to a wide variety of neurological problems. Also, it can enlarge the spleen and kidneys, as well as lower and destroy blood cell counts.
HLH is a genetic disease, which can be inherited in one of two ways: Autosomal Recessive or X-Linked. Autosomal Recessive is where the child has two copies of the abnormal gene, one from each parent. X-Linked is where HLH is passed to a male from the mothers X-chromosome, which carries the abnormal gene.
There are two types of HLH, familial and acquired. Familial HLH is shown in the first year of a person life, and affects 1 out of every 200,000 children. Acquired HLH occurs at any age, and is more common than familial HLH, because it can be triggered by an infection, virus, or cancer. The survival rate is only at 50%.
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