[Childhood hemophagocytic lymphohistiocytosis with pancytopenia triggered by leishmaniasis].
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Identifying the underlying cause of pancytopenia can be challenging, as there is a wide range of etiologies, including drugs, autoimmune conditions, malignancies, infections, hemophagocytosis and hereditary conditions. The authors present the case of a 3-year-old girl who was admitted for a prolonged febrile condition. Laboratory investigations revealed pancytopenia, increased inflammatory activity, and malignancy was excluded as the underlying cause of the symptoms. A diagnosis of hemophagocytic lymphohistiocytosis (HLH) was made on the basis of clinical signs and characteristic laboratory findings. She received combined immunosuppressive therapy as intravenous steroid, interleukin-1 antagonist, cyclosporine. Her condition stabilized and she was transferred to the Centre for Pediatric Immunology at the University of Milan. Additional investigations confirmed Leishmania infection. Therapy was supplemented with amphotericin B. The treatment resulted in a rapid improvement of the child’s condition and laboratory parameters. Persistent remission was observed even after the end of treatment. Our case study aims to highlight the rare pathology underlying pancytopenia. The treatment of this disease requires the use of different immunosuppressive therapies depending on the extent of the organ manifestations and the treatment of the underlying cause. Orv Hetil. 2025; 166(41): 1628–1633.