Abstract
Chronic or multi-lineage cytopenia should prompt enumeration of the double negative T cells (DNT) cells in peripheral blood and hence a suspicion of autoimmune lymphoproliferative syndrome (ALPS)
DNTs should be use in routine laboratory evaluation of ALPS patients together with the serum level of soluble Fas ligand, IL-10 and vitamin B12
Serum immunoglobulin levels help distinguish the ALPS due to FAS/FASL mutations from other subtypes
More than 90% of patients with FAS or FASL mutations exhibit hyper IgG and/or IgA
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Sharapova, S.O. (2019). Hemolytic Anemia and Lymphadenopathy. In: Rezaei, N. (eds) Pediatric Immunology. Springer, Cham. https://doi.org/10.1007/978-3-030-21262-9_86
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DOI: https://doi.org/10.1007/978-3-030-21262-9_86
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