SECONDARY HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS: A REPORT OF 10 CASES AND LITERATURE REVIEW
Sami Zarhloul*, Youssef Bighouab, Yousra Manar, Chaimae Dadi, Nouama Bouanani
ABSTRACT
Hemophagocytic lymphohistiocytosis (HLH) is a rare but life-threatening hyperinflammatory syndrome characterized by uncontrolled immune activation and excessive cytokine release. Secondary HLH is more commonly observed in adults and is typically triggered by infections, malignancies, or autoimmune diseases. The present study aimed to describe the clinical, biological, etiological, therapeutic, and prognostic characteristics of secondary HLH in a Moroccan tertiary care center. A retrospective descriptive study was conducted including 10 adult patients diagnosed with secondary HLH between January 2017 and December 2020. Clinical presentation, laboratory findings, underlying etiologies, treatment strategies, and outcomes were analyzed. Fever and splenomegaly were the most common clinical manifestations. Marked hyperferritinemia was observed in all patients, while cytopenias and hypertriglyceridemia were present in the majority of cases. Infectious etiologies accounted for 40% of cases, followed by malignancies (30%) and autoimmune diseases (20%). All patients received corticosteroid therapy, and etoposide was administered in 60% of cases. The overall mortality rate reached 60%. Secondary HLH remains a diagnostic and therapeutic emergency with a poor prognosis. Early recognition and prompt initiation of appropriate therapy are essential to improve patient outcomes.
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