What question did this study set out to answer?

To evaluate recent advances in identifying and managing hemophagocytic lymphohistiocytosis in adults.

April 4, 2026

Syndrome hémophagocytaire chez l’adulte

Key Points

To evaluate recent advances in identifying and managing hemophagocytic lymphohistiocytosis in adults.
Review of current literature on hemophagocytic lymphohistiocytosis.
Analysis of diagnostic approaches and treatment options according to severity.
Discussion on the role of anakinra in treatment.
A significant portion of patients remain without identified triggers for hemophagocytic lymphohistiocytosis.
Anakinra has shown promise in stabilizing patient conditions and facilitating diagnostic assessments.
Management strategies continue to rely heavily on expert opinion due to the varied nature of cases.

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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