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Can you fully recover from HLH?
All forms of HLH, including cases treated adequately, may have a high mortality rate. The long-term outlook ( prognosis ) of familial forms without treatment is poor, with a median survival of less than 2 months to 6 months after diagnosis. Even with treatment, only 21-26\% are expected to survive 5 years.
Is HLH treated with chemotherapy?
Secondary HLH can be effectively treated with chemotherapy and/or immunotherapy medications. Stem cell transplant is not necessary in most cases of secondary HLH. However, children without a known genetic cause for the HLH who experience progressive or recurrent disease are candidates for stem cell transplant.
Is HLH treatable?
Stem cell transplant can cure HLH in most cases. There is no way to prevent HLH, but as healthcare providers continue to learn more about it, treatment improves. Most children who are successfully treated go on to live normal lives.
How I treat relapsed HLH?
Systemic corticosteroids (typically dexamethasone) are the cornerstone of treatment. When more aggressive therapy is necessary, etoposide-based HLH-94 and HLH-2004 protocols are commonly used. In the HLH-94 regimen, corticosteroids, etoposide, cyclosporine A, and intrathecal therapy (methotrexate) are used.
Is HLH a malignancy?
HLH is not a malignant disorder. It is a highly stimulated defective severe inflammatory syndrome and tissue destruction resulting from an excessive immune activation and an absence of down-regulation of abnormally activated lymphocytes and macrophages [1].
How do you confirm HLH?
HLH can be diagnosed only with the proper tests. Blood tests include blood cell counts, liver function, infection studies, and markers of immune system activation such as ferritin and soluble IL-2 receptor levels. A bone marrow aspirate and biopsy may be performed to look for microscopic evidence of hemophagocytosis.
What is the survival rate of HLH?
If left untreated, patients with HLH survive for only a few months, due to progressive multi-organ failure. In 1994, the Histiocyte Society organized the first treatment protocol for HLH (HLH-94), which dramatically increased this survival rate to 54 percent with a median follow-up of six years [ 1,2 ].
How to diagnose HLH?
HLH can be diagnosed only with the proper tests. Blood tests include blood cell counts, liver function, infection studies, and markers of immune system activation such as ferritin and soluble IL-2 receptor levels. A bone marrow aspirate and biopsy may be performed to look for microscopic evidence of hemophagocytosis.
How do I treat HLH?
Treatment is very important for patients with HLH because the condition is life-threatening. The treatments that doctors use suppress the immune system. Patients are usually treated with steroids, chemotherapy (etoposide) and/or antibody therapies that target and destroy T cells (anti-thymocyte globulin / ATG as well as alemtuzumab).
How do we treat HLH?
Chemotherapy (cancer drugs)