Hemophagocytosis phenomenon in hemophagocytic lymphohistiocytosis
DOI:
https://doi.org/10.24197/8w95gy20Keywords:
lymphohistiocytosis, hemophagocyitic, macrophageAbstract
Hemophagocytic lymphohistiocytosis (HLH) is a severe hyperinflammatory syndrome caused by uncontrolled activation of macrophages and cytotoxic T lymphocytes, leading to a “cytokine storm” and subsequent multiorgan damage. It is classified into primary HLH (genetic origin, involving defects in NK cells and CD8+ T cells) and secondary HLH, which is triggered by infections, hematologic malignancies, or autoimmune diseases.
Clinically, HLH presents with persistent fever, cytopenias, liver dysfunction, and in severe cases, neurological involvement. Diagnosis is established by meeting at least five out of eight clinical and laboratory criteria, including hyperferritinemia, hemophagocytosis in bone marrow, and impaired NK cell activity.
Treatment for primary HLH involves immunochemotherapy and hematopoietic stem cell transplantation. In secondary HLH, management focuses on treating the underlying cause and controlling inflammation with immunosuppressive or emerging biologic therapies. Early recognition is critical, as mortality exceeds 50% without appropriate treatment.
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References
Ramos-Casals M, Brito-Zeron P, Lopez- Guillermo A, Khamashta MA, Bosch X. Adult haemophagocytic syndrome. Lancet. 2014; 383(9927):1503-1516
Daver N, McClain K, Allen CE, et al. A consensus review on malignancy-associated hemophagocytic lymphohistiocytosis in adults. Cancer. 2017;123(17):3229-3240
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