Genetic errors of immunity distinguish pediatric nonmalignant lymphoproliferative disorders.

ABSTRACT:

Background

Pediatric nonmalignant lymphoproliferative disorders (PLPDs) are clinically and genetically heterogeneous. Long-standing immune dysregulation and lymphoproliferation in children may be life-threatening, and a paucity of data exists to guide evaluation and treatment of children with PLPD.

Objective

The primary objective of this study was to ascertain the spectrum of genomic immunologic defects in PLPD. Secondary objectives included characterization of clinical outcomes and associations between genetic diagnoses and those outcomes.

Methods

PLPD was defined by persistent lymphadenopathy, lymph organ involvement, or lymphocytic infiltration for more than 3 months, with or without chronic or significant Epstein-Barr virus (EBV) infection. Fifty-one subjects from

SUBMITTER: Forbes LR 

PROVIDER: S-EPMC8795244 | biostudies-literature | 2022 Feb

REPOSITORIES: biostudies-literature