n HOW IS HLH TREATED? • The treatment for HLH involves turning down the body’s infec- tion-fighting response, a process is called immunosuppression . The most commonly used medications are dexamethasone, etoposide, and cyclosporine. Sometimes parents are confused when chemotherapy is used to fight HLH. However, in this situa- tion, chemotherapy is being used for its well-known side effect of suppressing the immune system, which is what patients with HLH need—a way to turn down or regulate their immune system.
• The only curative treatment for patients with the inherited form of HLH is to replace their defec- tive immune system with a healthy one through the process of stem cell transplant. Patients with the acquired form of HLH may also need a stem cell transplant if they do not get better after several weeks of chemotherapy.
n HOW LONG WILL MY CHILD’S THERAPY LAST? The initial treatment phase lasts for 8 weeks. At that time, if your child has the inherited form of the disease, or if blood tests show that NK cells still are not working correctly, then your child will continue chemotherapy while your doctors make plans for stem cell transplant as soon as a suitable donor can be found. After the initial 8 weeks of chemotherapy, if your child has the acquired form of HLH, and the special blood tests show that NK cells are working normally again, your doctors may stop chemotherapy and continue to watch for improvement by checking blood tests. n HOW SUCCESSFUL IS THE TREATMENT? Successful treatment of HLH depends on making the diagnosis quickly so that therapy can be started as soon as possible and the disease can be brought under control. After 8 weeks of treatment with dexa- methasone and etoposide, about 75% of children diagnosed with HLH will be in remission, based on the results of the first international clinical trial for treatment of HLH (Filipovich, 2008). Overall survival rates for HLH are 55%. The survival rates for the inherited form of HLH have increased to 60% with early treatment and use of stem cell transplant (Gupta & Weitzman, 2010). Clinical trials are currently evaluating ways to improve early response without causing intolerable side effects. n ARE THERE ANY NEW TREATMENTS? According to the well-established treatment regimen of the Histiocyte Society, the best known treatment for HLH involves suppression of the immune system with etoposide, dexamethasone, and cyclosporine. Stem cell or bone marrow transplants are used for patients with the inherited form of the disease. Other treatment regimens have been reported using different combinations of immunosuppressive medica- tions. Alemtuzumab (Campath ® ) is being investigated as a possible treatment for patients who do not respond to established therapies.
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