I think I found what the disease was, though I honestly have no idea why they would test this rather than XLA. There’s a condition called Transient Hypogammaglobulinemia of Infancy. It presents w/low immunoglobulin levels post 6 months and can present w/small lymph nodes and tonsils in infancy BUT w/o any other findings of primary immunodeficiency including decreased counts.
Here’s an article about it: https://primaryimmune.org/about-primary-immunodeficiencies/specific-disease-types/transient-hypogammaglobulinemia-of-infancy/
You definitely don’t need to know the disease to get the correct answer since the link of lack of immunoglobulins would clue you into the lack of germinal centers, but I think this is more likely than XLA since every source I read implies that B cell counts are near 0 in the classic presentation (unless I’m missing a reason why leukocyte count w/diff wouldn’t show a significant decrease in lymphocytes due to near-zero B cells). Just wanted to put this here in case other people later came wondering, though I may still be wrong.
I made a lucky guess and chose this but I don’t think for the right reasons. I thought maybe he has BTK deficiency/Bruton’s Agammaglobinemia. But, now that I am going over it I wasn’t sure. Would that show a normal leukocyte differential? Is it CVID? Didn’t think CVID would have absent germinal centers in lymph nodes. What else could this be?
Male child, recurrent infections, no mature B cells = Bruton disease (X-linked agammaglobulinemia)
Common variable immunodeficiency - Defect in B-cell differentiation. Cause unknown in most cases. May present in childhood but usually diagnosed after puberty. Increased risk of autoimmune disease, bronchiectasis, lymphoma, sinopulmonary infections Decreased plasma cells, Decreased immunoglobulins
submitted by hipster_do(8)
I’m going to say it’s X linked agammaglobulinemia rather than SCID, but the difference between these two are tiny but this is why I think it’s the former:
SCID should be immediately because they just don’t have the IL2 receptors. CVID shows up when they’re 20-40 years old. You get absent germinal centers in both. No mention of absent thymic shadow which is in SCID.