Severe peripheral blood lymphopenia without NK cell cytotoxicty deficiency is the rule in adult acquired HLH
نویسندگان
چکیده
Introduction Hemophagocytic lymphohistiocytosis (HLH) is characterized by hypercytokinemia and hemophagocytosis due to abnormal activation and proliferation of T lymphocytes and macrophages. Inherited forms are due to gene defects affecting CD8 and NK lymphocyte cytotoxicity, whereas in acquired forms complicating rheumatic, infectious or neoplastic diseases, lymphocyte subpopulation and function profiles remain poorly studied.
منابع مشابه
Biology and Treatment of Hemophagocytic Lymphohistiocytosis
Hemophagocytic lymphohistocytosis (HLH) is a hyperinflammatory syndrome that occurs at all ages and is characterized by high levels of cytokines, secreted by activated T-lymphocytes and macrophages. All symptoms and laboratory changes can be explained by organ infiltration by these cells and hypercytokinemia. HLH occurs as an inherited form (genetic, primary HLH) with mutations primarily in the...
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