Mazloum, Y., El Beheiry, A., Abdel-Maksoud, M., Ali Negm, B. (2024). MAGNETIC RESONANCE IMAGING PATTERNS IN PEDIATRIC CENTRAL NERVOUS SYSTEM HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS. ALEXMED ePosters, 6(3), 2-3. doi: 10.21608/alexpo.2024.300166.1876
Yasser Mazloum; Ahmed Adel Hassan El Beheiry; Marwa Saeed Abdel-Maksoud; Basant Gamal Ali Negm. "MAGNETIC RESONANCE IMAGING PATTERNS IN PEDIATRIC CENTRAL NERVOUS SYSTEM HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS". ALEXMED ePosters, 6, 3, 2024, 2-3. doi: 10.21608/alexpo.2024.300166.1876
Mazloum, Y., El Beheiry, A., Abdel-Maksoud, M., Ali Negm, B. (2024). 'MAGNETIC RESONANCE IMAGING PATTERNS IN PEDIATRIC CENTRAL NERVOUS SYSTEM HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS', ALEXMED ePosters, 6(3), pp. 2-3. doi: 10.21608/alexpo.2024.300166.1876
Mazloum, Y., El Beheiry, A., Abdel-Maksoud, M., Ali Negm, B. MAGNETIC RESONANCE IMAGING PATTERNS IN PEDIATRIC CENTRAL NERVOUS SYSTEM HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS. ALEXMED ePosters, 2024; 6(3): 2-3. doi: 10.21608/alexpo.2024.300166.1876
MAGNETIC RESONANCE IMAGING PATTERNS IN PEDIATRIC CENTRAL NERVOUS SYSTEM HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS
1Radiodiagnosis and Intervention, Medicine, Alexandria, Alexandria, Egypt
2Department of Radiology, Faculty of Medicine, Alexandria University, Egypt
3Department of pediatrics, Faculty of Medicine, Alexandria University
4Department of Radiodiagnosis and Intervention, Faculty of Medicine, Alexandria University
Abstract
Introduction HLH is a rare, life-threatening pediatric condition characterized by uncontrolled hyperinflammation caused by various hereditary or acquired immune deficiencies. It is classified into two subgroups: Primary HLH including familial HLH (FHLH) and immunological deficits and secondary HLH which can be caused by infection, cancer, rheumatology, or iatrogenic factors (transplantation, immunological suppression, immune stimulation). HLH is always fatal unless treatment results in a partial or total clinical response. Chemo-immunotherapy with steroids, etoposide, and cyclosporine, as well as rabbit antithymocyte globulin, cyclosporine, and prednisone, can be used with or without intrathecal methotrexate. So far, the only curative therapy strategy is allogeneic hematopoietic stem cell transplantation. Central nervous system (CNS) involvement, has been documented as an independent risk factor for mortality in children with HLH. Clinically, due to the rarity of pathological studies of HLH, making the diagnosis of CNS involvement difficult, clinicians must rely on clinical manifestations combined with imaging and laboratory tests to confirm such diagnosis.
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