Undiscovered (or too late) and lead to fatality for the patient. Consequently, Hematologists and Oncologists who treat cancerīecause the disease is relatively new from a research perspective (1985) andĬauses as well as symptoms are still being discovered, many cases go Over the years cancer treatments have been used in Survival rates after HCT have improved from 50-70% up to 90% in experienced HCT Widespread use of Reduced Intensity Conditioning (RIC) protocols accompanied byĬareful selection of donor and stem cell product to be used - long term Recurrent episodes are also considered for BMT. Patients without a known genetic cause who experience progressive HLH or Genetic defects will require a hematopoietic cells transplant (BMT) to cure Ultimately, children and young adults who have HLH on the basis of known Well to steroids initially, many patients suffer from progressive illness whichĬan lead to death if appropriate immune suppressive treatment is not begun in While some patients experience HLH symptoms which can wax and wane or respond The high cytokine levels, resulting in the low blood counts. Is an abnormal consumption of blood cells by macrophages which are turned on by Hemophagocytosis, the process for which the disorder is named. This so-called "hypercytokinemia" is potentially damaging Intense immune response results in prolonged and highly elevated levels ofĬytokines (molecules which, in minute quantities, normally regulate immuneįunctions). Immune response involving T lymphocytes and macrophages. The symptoms of HLH result from an overactive and poorly controlled Manner (where both parents are carriers) or in an x-linked manner affecting FHLH can be inherited in either an autosomal recessive To date, 7 genetic causes of FHLH have been discovered, Redness, heat, pain and malfunction/damage of organs when they attack andĪccumulate in healthy lymph tissue (ed: Liver, Spleen, Lymph Histiocytes and lymphocytes, which are white blood cells that normallyĬontrol infections, but when overactivated can cause swelling, With infections, autoimmune conditions or cancer. Genetic forms (Familial HLH or FHLH) or acquired forms which may be associated At least half of the patients with HLH will also experience inflammation of the brain which can lead to a wide variety of neurological problems. Common symptoms are fevers, enlarged spleen, low blood counts and liver abnormalities. It affects people of all ages and ethnic groups. HLH stands for Hemophagocytic Lymphohistiocytosis and is a life-threatening immunodeficiency.
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