Hematopoietic cells are instrumental in generating and propagating protective inflammatory
responses to infection or injury. However, excessive inflammation contributes to many …

Inborn errors of immunity (IEIs) are a group of conditions whereby parts of the immune system
are missing or dysfunctional. Once thought to primarily be a pediatric disorder, it is now …

Renal disease is an underappreciated complication of inborn errors of immunity (IEIs). Chronic
kidney disease (CKD) is associated with significant morbidity and mortality in the general …

E1404 CMAJ| NOVEMBER 9, 2020| VOLUME 192| ISSUE 45 hasten reversal of the cerebral
vasoconstriction, we admitted the patient and started treatment with verapamil immediate …

Background C1 inhibitor (C1-INH) and complement 4 (C4) have historically been referred to
as positive acute phase reactants, however this has never been evaluated in hereditary …

A young woman with persistent EBV viremia and lymphocytosis had an abnormal CD4- T
cell population with aberrant loss of CD7. She had a diagnosis of chronic active EBV (CAEBV), …

Methods Charts of patients seen at the Saint Paul’s Hospital Primary Immunodeficiency
Transition Clinic from February 2018 to June 2019 were retrospectively reviewed. 24 patients …

Germline pathogenic mutations in the GATA2 gene, a critical transcription factor in hematopoietic
and neurologic development, are known to cause various syndromes characterized by …

Background Immunoglobulin replacement therapy is standard of care for patients with
primary humoral immunodeficiency [1]. Compared with intravenous immunoglobulin (IVIG), …

Immunophenotyping of our patient further supported a diagnosis of CTLA-4 insufficiency,
demonstrating absolute CD3 SP+ sp T cell lymphopenia with normal B and NK (natural killer) …