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Med. Clin. North Am. · Jan 2024
ReviewHemophagocytic Lymphohistiocytosis in Adolescents and Young Adults: Genetic Predisposition and Secondary Disease.
- Alejandra Escobar Vasco, Julie-Ann Talano, and Larisa Broglie.
- Medical College of Wisconsin, 8701 Watertown Plank Road, MFRC 3018, Milwaukee, WI 53226, USA; Division of Hematology/Oncology/Blood and Marrow Transplantation, Department of Pediatrics, Medical College of Wisconsin, 8701 Watertown Plank Road, MFRC 3018, Milwaukee, WI 53226, USA.
- Med. Clin. North Am. 2024 Jan 1; 108 (1): 189200189-200.
AbstractHemophagocytic lymphohistiocytosis (HLH) is a disorder of impaired immune regulation resulting in hyperinflammation that is ultimately fatal if not treated. HLH is categorized into familial disease, caused by genetic mutations affecting the function of cytotoxic T lymphocytes and natural killer cells, and secondary disease, triggered by infections, malignancies, rheumatologic disorders, or immune deficiency. Adolescent and young adults with HLH represent a unique population with specific diagnostic challenges. Here we review the diagnostic criteria, possible etiologies, pathophysiology, and management of HLH with focus on the adolescent population.Copyright © 2023 Elsevier Inc. All rights reserved.
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