Histiocytic sarcoma progressing from follicular lymphoma and mimicking acquired hemophagocytic lymphohistiocytosis

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Hereditary and acquired hemophagocytic lymphohistiocytosis.

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Hemophagocytic lymphohistiocytosis (HLH) is a potentially life-threatening clinical syndrome caused by uncontrolled activation of lymphocytes and histiocytes resulting in high levels of cytokines. Acquired HLH occurs in autoimmune, inflammatory, infectious, and immunosuppressive disorders. Prompt identification and treatment of an underlying triggering cause improves clinical outcome.

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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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ژورنال

عنوان ژورنال: Annals of Hematology

سال: 2020

ISSN: 0939-5555,1432-0584

DOI: 10.1007/s00277-020-04190-4