Abstract
Secondary hemophagocytic lymphohistiocytosis in diffuse large-cell B lymphoma: a case-report Secondary hemophagocytic lymphohistiocytosis (sHLH) is a rare, life-threatening and often misunderstood condition characterized by uncontrolled immune activation. Patients typically present with a pentad of symptoms including fever, hepatosplenomegaly, cytopenias affecting at least 2 of 3 cell lines, and elevated transaminase and ferritin levels. Familial hemophagocytic lymphohistiocytosis (FHL), the primary form, mainly occurs in pediatric age. The underlying pathogenesis is associated with genetic defects affecting cellular immunity (CD8+ T-lymphocytes and natural killer cells). The secondary forms (sHLH) mainly occur in adulthood and are linked to underlying processes such as autoimmune diseases, malignancies or infections. Recent revisions of the diagnostic criteria, including the H-score, offer valuable guidance for clinical decision-making. Early detection is crucial, followed by a prompt initiation of immunosuppressive therapies, such as corticosteroids, methotrexate, etoposide or eventually a hematopoietic stem cell transplantation (HSCT). This case-report illustrates a 79-year-old woman with sHLH secondary to an underlying B-cell lymphoma, underscoring the significance of recognition, diagnosis and treatment.
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