Identical phenotype in patients with somatic and germline CD95 mutations requires a new diagnostic approach to autoimmune lymphoproliferative syndrome.

Rössler, Jochen; Enders, Anselm; Lahr, Georgia; Heitger, Andreas; Winkler, Karl; Fuchs, Hans; Kopp, Matthias; Niemeyer, Charlotte et al. · J Pediatr · 2005

case_report · Level V

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Abstract

In a patient with a somatic mutation in the CD95 gene, the long-term evolution of the clinical phenotype was indistinguishable from that of patients with autoimmune lymphoproliferative syndrome caused by germline CD95 mutations. A new diagnostic algorithm for autoimmune lymphoproliferative syndrome is suggested incorporating studies on sorted TCRalpha/beta+CD3+CD8-CD4- T cells.

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