The X-linked lymphoproliferative (XLP) syndrome is classically associated with overwhelming infection with which organism?

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Question 1 of 5

The X-linked lymphoproliferative (XLP) syndrome is classically associated with overwhelming infection with which organism?

Correct Answer: A

Rationale: XLP is classically linked to severe, often fatal Epstein-Barr virus infections due to defective immune regulation.

Question 2 of 5

An 8-month-old infant presented with chronic diarrhea, persistent right ear discharge, oral candidiasis responding poorly to antifungal drugs, and failure to thrive. On examination the infant has exfoliative erythroderma, lymphadenopathy, and hepatosplenomegaly. Peripheral blood showed eosinophilia and elevated IgE. The parents are young first cousin and he is their first baby. Of the following, the MOST likely diagnosis is

Correct Answer: C

Rationale: Omenn syndrome, a form of SCID, presents with erythroderma, eosinophilia, and elevated IgE due to consanguinity-related genetic defects.

Question 3 of 5

Viral infections associated with neutropenia include

Correct Answer: D

Rationale: Hepatitis B is known to cause neutropenia, unlike the other listed viral infections which are less commonly associated.

Question 4 of 5

When we inhibit alternative pathway convertase, all of the following would decrease EXCEPT

Correct Answer: C

Rationale: The alternative pathway involves C3b and does not form C4b2b, which is specific to the classical/lectin pathways, so inhibiting it would not affect C4b2b levels.

Question 5 of 5

Which of the following is a soluble pattern recognition receptor

Correct Answer: C

Rationale: Pentraxin 3 is a soluble pattern recognition receptor in the innate immune system, unlike the others which are membrane-bound or intracellular.

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