Primary immunodeficiencies (PIDs), a heterogenous group of inborn errors of immunity, are
predetermined at birth but may evolve with age, leading to a variable clinical and laboratory …

Peripheral T cell lymphomas (PTCLs) are a heterogeneous group of orphan neoplasms.
Despite the introduction of anthracycline-based chemotherapy protocols, with or without …

Biallelic mutations in the genes encoding CD27 or its ligand CD70 underlie inborn errors of
immunity (IEIs) characterized predominantly by Epstein-Barr virus (EBV)-associated immune …

Primary immunodeficiencies and immune dysregulatory disorders (PIDDs; now referred to
as inborn errors in immunity) are rare disorders with a prevalence of 41. 4 or 50.5 per …

Inborn errors of immunity (IEI), formerly known as primary immunodeficiency disorders
(PIDs), are inherited disorders caused by damaging germline variants in single genes …

Ataxia-telangiectasia (AT) is an autosomal-recessive disorder caused by pathogenic
variants (PVs) of the ATM gene, predisposing children to hematological malignancies. We …

Simple Summary Inborn Errors of Immunity (IEI) are a heterogeneous group of disorders
characterized by a defect in the function of at least one, and often more, components of the …

Common variable immunodeficiency (CVID) represents the largest group of primary
immunodeficiencies that may manifest with infections, inflammation, autoimmunity, and …

The thymus is critical for central tolerance and diverse T-lymphocyte repertoire development,
to provide lifelong defense against pathogens while maintaining self-tolerance. Peak thymic …

Background Survival of children and adolescents with high-risk, mature B-cell non-Hodgkin
lymphoma is improved by the addition of rituximab to chemotherapy. The effect of rituximab …