n WHO GETS HLH? HLH can occur at any age from birth to adulthood, but most patients are younger than 15 years of age. About 1 out of every 100,000 children younger than the age of 15 years will be diagnosed with HLH. Boys and girls are diagnosed with HLH in equal numbers. The inherited form of the disease usually occurs in children younger than the age of 2 years.
n IS HLH INHERITED? Yes, there is an inherited form of HLH. Researchers have discovered five different abnormal genes that can result in HLH. When blood tests show the presence of one of these gene mutations in a child with HLH, the brothers and sisters also may be at risk for developing HLH. However, not all forms of the disease are inherited (see below).
n IS HLH A FORM OF CANCER? No, HLH is a disorder of the immune system. It is not cancer, but chemotherapy is often used to treat HLH because one of the main side effects of chemotherapy is its ability to decrease the body’s infection- fighting response.
n WHAT ARE SOME OF THE SIGNS AND SYMPTOMS OF HLH? Children with HLH are usually very sick at first. The symptoms of HLH are the same for both the inherited and the acquired form. They include
• fevers that last for a long time • enlarged liver and/or spleen • rash • increased bleeding and bruising • abnormal blood tests – low blood counts (white blood cells, hemoglobin, and/or platelets) – low fibrinogen and/or high triglycerides – high ferritin • abnormal brain function causing increased irritability.
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