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Microbiology · Immunology: common variable immunodeficiency
Sample itemA 28-year-old man has had recurrent sinusitis and pneumonia since age 22 and now has chronic diarrhoea with Giardia in the stool. Serum IgG, IgA and IgM are all low, antibody response to vaccines is absent and non-caseating granulomas are found in the lung. Flow cytometry is most likely to show which pattern, distinguishing this disorder from X-linked agammaglobulinaemia?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
D. Near-normal CD19-positive B cells that fail to differentiate into plasma cells
Common variable immunodeficiency usually presents in the second to fourth decade with hypogammaglobulinaemia, poor vaccine responses, giardiasis, autoimmunity and granulomatous disease. B cells are present in normal or slightly reduced numbers but do not mature into antibody-secreting plasma cells. In X-linked agammaglobulinaemia, B cells are almost absent and presentation is in infancy.
Option by option
A. Absent CD19-positive B cells with a BTK gene mutation
Absent B cells from a BTK mutation describe X-linked agammaglobulinaemia, which presents in a boy in infancy.
B. Normal B cells with absent CD40 ligand on activated T cells
CD40 ligand deficiency causes hyper IgM syndrome, in which IgM is raised rather than low.
C. Absent CD3-positive T cells with adenosine deaminase deficiency
Absent T cells with ADA deficiency describe a form of severe combined immunodeficiency presenting in the first months of life.
D. Near-normal CD19-positive B cells that fail to differentiate into plasma cells (correct)
Near-normal numbers of B cells that cannot become plasma cells fit the adult onset, low immunoglobulins of all classes and granulomas.
Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.
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