Abstract
Autoimmune lymphoproliferative syndrome is a rare disorder arising from a mutation of the Fas gene, which encodes a receptor that induces cell death trigged by FasL and belongs to the tumor necrosis factor receptor superfamily. Patients with ALPS have a defect in the Fas–FasL apoptotic pathway, leading to chronic lymph proliferation, autoimmune manifestations, and a propensity to develop malignancies, particularly B-cell lymphomas. 18F-FDG–PET/CT has several roles to play in patients with ALPS, e.g., ruling out a diagnosis in patients with a suspected malignancy and monitoring the response to treatment.
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© 2014 Springer-Verlag Italia
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Cistaro, A. (2014). Autoimmune Lymphoproliferative Syndrome. In: Cistaro, A. (eds) Atlas of PET/CT in Pediatric Patients. Springer, Milano. https://doi.org/10.1007/978-88-470-5358-8_32
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DOI: https://doi.org/10.1007/978-88-470-5358-8_32
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