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Microbiology · Immunology: leukocyte adhesion deficiency
Sample itemA 2-month-old infant had delayed umbilical cord separation at 5 weeks and now has omphalitis and a skin infection without pus. The neutrophil count is 40,000 per microlitre. Which defect is most likely?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
B. Deficiency of CD18 beta-2 integrins on leukocytes
In leukocyte adhesion deficiency type 1, absent CD18 prevents firm adhesion and transmigration of neutrophils into tissues. Cells accumulate in blood, giving marked neutrophilia, while infected sites lack pus. Delayed cord separation beyond 3 weeks is the classic early clue.
Option by option
A. Deficiency of the oxidase that generates superoxide
Oxidase deficiency allows migration but impairs killing, and cord separation is normal.
B. Deficiency of CD18 beta-2 integrins on leukocytes (correct)
Correct. Without CD18 integrins, neutrophils cannot leave the vessels and pus does not form.
C. Failure of immunoglobulin class switching
Class switching failure lowers IgG, IgA and IgE and does not cause neutrophilia.
D. Deficiency of the terminal complement components
Terminal complement deficiency causes recurrent Neisseria infection.
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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