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NBME 22 Answers

nbme22/Block 3/Question#33 (39.5 difficulty score)
A 22-year-old man comes to the physician ...
47,XXY🔍
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 +5  upvote downvote
submitted by aesalmon(79),
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dLygie elcl yaelhprps,ia ohsudl olas ees osibfsir nda yltiaanzhino fo het sbtlueu tub im' nto nseige ..it. /)_(¯¯ツ_

meningitis  I think its there.. I thought it was the lighter red/pink material (hyaline material) And I thought the white streaks were the fibrosis like "streaked ovaries"in Turners. +  

 +5  upvote downvote
submitted by step1soon(43),

Testicular biopsies of adult males with Klinefelter syndrome are characterized by extensive fibrosis and hyalinization of the seminiferous tubules and hyperplasia of the interstitium; however, the tubules may show residual foci of spermatogenesis. Histologic findings may include small, firm testes with seminiferous tubular hyalinization; sclerosis; and atrophy with focal hyperplasia of mostly degenerated Leydig cells

http://www.pathopedia-india.com/Klinefelter_Syndrome.htm




I kind of reasoned through this by thinking that if they said "22 year old man" that means there has to be a Y in there in order for the person to be presumed male. That left options: 45X/46XY, 46XY, and 47XXY. Beyond that I thought that the picture didn't look normal (couldn't make out tubules or individual cells), so it couldn't be 46XY.

I figured 45X/46XY is a mosaic Turner, and I have no idea what a person with that genotype would look like, nor whether they would have testes, but I know that 47XXY would have testes because SRY is on chromosome Y which makes testes determining factor.




 +2  upvote downvote
submitted by waterloo(54),

45, X / 46, XY is a bit tricky. Not having too much time to think, I almost went for it thinking there is increased risk for gonadoblastoma with 45X/46XY mosaicism - so maybe that's what they are going for? But what I realized after a couple NBME's, if they don't mention something about the body habitus (like here they said male), it's less likely this patient has Turner Syndrome.

We're just medical students, not pathologists. So I think if you just say okay, male so can't be 46 XX or 47 XXX. Then also probably not mosaicism cause of what I mentioned above. You are left with normal 46 XY or 47 XXY - and which is more likely to have fertility issues?




 +1  upvote downvote
submitted by bubbles(63),
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saH dnoyayb onfdu a oogd eantixlpaon for siht lohtoys?gi I gunneilye vhea on deia whta mI' olkogin t.a

meningitis  This is common in Klinefelter.. think of the equivalent of Streaked ovaries seen in Turners. White streaks, red/pink material of hyaline, and hyperplasia of Leydig cells. Just remember: It doesn't look like normal structured testicle histology (No organized seminiferous tubules with Sertoli cells around) +9  
niboonsh  https://www.pathologyoutlines.com/topic/testisklinefelter.html these pictures are kinda similar +2