HEMOPHAGOCYTIC LYMPHOHISTOCYTOSIS: A CASE REPORT
DOI:
https://doi.org/10.70194/amg-5564کلمات کلیدی:
Hemophagocytic-lymphohistocytosis، hemophagocytic syndrome ،macrophage activation syndrome.چکیده
Background: Hemophagocytic lymphohistiocytosis (HLH) or hemophagocytic syndrome is a rare, life-threatening hyperinflammatory syndrome characterized by excessive, overactive, and abnormal immune activation. It can be either primary (genetic) or secondary to infections, malignancies, or autoimmune diseases. Early diagnosis and treatment are crucial but often challenging due to the nonspecific clinical presentation.
Case presentation: We report a case of 22 a 22-year-old male who presented with persistent fever, hepatosplenomegaly, cytopenias, and elevated ferritin levels. Laboratory findings revealed hypertriglyceridemia, elevated liver enzymes, and high serum ferritin, raising suspicion for HLH. Bone marrow biopsy confirmed hemophagocytosis. The patient was managed with dexamethasone and etoposide according to the HLH protocol, with clinical improvement observed within 12 weeks.
Conclusion: This case highlights the importance of early recognition of HLH and the use of HLH diagnostic criteria for timely treatment. Due to its rapid progression and high mortality, clinicians should maintain a high index of suspicion for HLH in patients with unexplained systemic inflammatory symptoms and cytopenias.
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