​Pathology - Common Variable Immunodeficiency

​Pathology - Common Variable Immunodeficiency
Caused by a deficiency in intrinsic B cells that stops them from maturing into plasma cells that release antibodies, which leads to insufficient secreted antibody production.
Peak onset typically occurs in late adolescence, after the ages of 1 and 5.

Pathology
 Histiocytes encircle noncaseating granulomas in the skin, lungs, liver, and spleen.
increased frequency of autoimmune disorders, sprue-like GI condition, and recurring pyogenic infections, which typically impact the respiratory system.


Skin cancer, stomach carcinomas, and B-cell neoplasms are among the complications that may arise.
The results of the lab analysis show that there is a decline in IgG levels over time, affecting all antibody classes; functional antibody responses to protein antigen vaccinations are either missing or reduced; and the total number of B cells in peripheral blood is normal.

routine IVIG treatment; infection management.

The most prevalent primary immunodeficiency disease, selective lgA deficiency, is characterized by a lack of serum IgA, maybe as a result of an isotype switching problem. It manifests as lung and sinus infections, GI tract infections leading to chronic diarrhea, food allergies and other allergy illnesses (such as rhinitis or atopic dermatitis), and anaphylaxis following blood transfusions.