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The challenge of early diagnosis of autoimmune lymphoproliferative syndrome in children with suspected autoinflammatory/autoimmune disorders

Rheumatology May 01, 2021

Mendonça LO, Matucci-Cerinic C, Terranova P, et al. - In the present study, the researchers tested the usefulness of an extended panel of lymphocyte subsets (LS) in combination with Oliveira’s diagnostic criteria for the identification of autoimmune lymphoproliferative syndrome (ALPS) in children referred to a pediatric rheumatology center. Patients referred to the Rheumatology Unit for an autoimmune or autoinflammatory condition from 2015 to 2018 were studied retrospectively. Two hundred sixty-four patients were included in the study. Oliveira’s required criteria are beneficial for the early suspicion of ALPS. TCRαβ+B220+ lymphocytes should be included in the diagnostic work-up of patients admitted to a pediatric rheumatology unit with a suspected autoimmune or autoinflammatory condition, as they may help detect ALPS early.

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