This is a serious inherited immunodeficiency disorder where the body’s immune system fails to fight infections effectively. The condition is more frequently observed in males. It can be inherited through either autosomal recessive or X-linked patterns.
In Glanzmann–Riniker syndrome, the blood contains virtually no B-lymphocytes (a type of white blood cell) and, as a result, antibodies (proteins produced by B-lymphocytes to destroy invading microorganisms) fail to form.
This condition becomes apparent early in infancy, as affected children fail to thrive. It is marked by the absence of the thymus, a gland that plays a role in the immune system, making individuals susceptible to bacterial and viral infections. Symptoms and signs of this condition include watery diarrhea and the formation of lung abscesses. Unfortunately, children with this condition typically do not survive beyond their first two years of life.