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Fever of Unknown Origin in Adult Caused by Hemophagocytic Lymphohistiocytosis: A case Report and Review of the Literature

Author(s): Mendez GD, Vivas M, Rodriguez-Pascual J

Hemophagocytic lymphohistiocytosis (HLH), also known as hemophagocytic syndrome, is a very uncommon and life-threatening hematologic disorder that occurs as a primary or acquired disease. Secondary HLH usually appears in the setting of infections, underlying rheumatologic disorders or lymphoid malignancies. Clinically the syndrome is characterized by fever, multiple cytopenias, hepatosplenomegaly and activated macrophages in hematopoietic organs. We describe a rare case of HLH in the context of abdominal pain and fever of unknown origin, dramatic worsening and, finally, successful evolution with immunosuppressive therapy. In addition, we review the available literature on this topic.

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