Author: George, Melissa R
Title: Hemophagocytic lymphohistiocytosis: review of etiologies and management Document date: 2014_6_12
ID: 3frxd1c1_28
Snippet: George gene mutations responsible. The genetic abnormalities identified in these syndromes are outlined in Table 7 . Generally, the forms of HLH associated with infants and young children are caused by defects in immune regulation, such as mutations in genes controlling the function of cytotoxic T-lymphocytes and NK-cells. With older children and adults, HLH is more likely to be secondary to infection, malignancy, or autoimmune disease. While rar.....
Document: George gene mutations responsible. The genetic abnormalities identified in these syndromes are outlined in Table 7 . Generally, the forms of HLH associated with infants and young children are caused by defects in immune regulation, such as mutations in genes controlling the function of cytotoxic T-lymphocytes and NK-cells. With older children and adults, HLH is more likely to be secondary to infection, malignancy, or autoimmune disease. While rare, some familial cases have been undetected until adulthood. Even children with a defined genetic cause of HLH often have a secondary assault such as infection that triggers HLH, in keeping with the two-hit hypothesis required for the development of many diseases.
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