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[Hemophagocytic lymphohistiocytosis in adults]
Mireille Gauthy1, Mhedi Belkoniene1, Sabine Ammann1
1Service d'immunologie et allergologie, Institut central des hôpitaux, Hôpital du Valais, 1950 Sion.
Abstract:
Hemophagocytic lymphohistiocytosis is a diagnostic and therapeutic emergency characterized by a cytokine storm. This hyperinflammation results from a complex interaction between innate immunoregulation and the environment. An etiological assessment must be conducted simultaneously with hyperinflammation treatment. No cause is found in nearly one-third of patients despite a thorough assessment. Anakinra limits interference with additional tests and provides a decisive time frame for identifying triggers. Treatment choices are largely based on expert opinion. This review summarizes recent advances regarding hemophagocytic lymphohistiocytosis in adults, proposes a diagnostic approach and stratified management according to severity and etiology.
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