A diagnosis of CVID is reserved for persons with
A diagnosis of CVID is reserved for persons with an undefined B-cell dysfunction.
Autoimmunity is found in about 25% of patients and to date the pathogenesis of the autoimmunity is not understood – immune thrombocytic anemia and hemolytic anemia
Lymphoma is a common cause of death in patients with CVID.\
1) We don't give blood transfusions to someone who IgA deficient because they may have anti-IgA antibodies but then why do we give IVIg to treat hyper IgM syndrome? Wouldn't they also make anti-IgA antibodies? – it is more common for CVID to just have low serum IgA levels rather than complete deficiency but if you do encounter a patient with complete IgA deficiency then there is a very real chance of an anti-IgA antibodies – in this situation IVIG administration benefits may still outweigh the chance of reaction but probably best determined on a case by case basis
This is one of the diseases that has delayed diagnosis –
Be very careful prescribing immunosuppressants to immune defieicent patients!