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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.

Severe peripheral blood lymphopenia without NK cell cytotoxicty deficiency is the rule in adult acquired HLH