.......part 11 "catch" in the diagnosis - roentgen

[ArchivalUser]

i ve changed now .........i agreewith dr.plaacebo 3333

[ArchivalUser]

Yo...roentgen,
Sorry had a brief nervous breakdown & was off 4 a while....
Yeah I'm the same placebo....

[ArchivalUser]

pLATELETS ARE NORMAL number, doesnt mean their function is normal. Cryoprecipitate contains factors 8,9,fibrinogen: so we can exclude 1,2,3,and 7,6 is nonsense,
we have left 4 and 5 possible correct. 5 may not be correct as the bleeding is for a long time and is not represented just by pethechia and the fact that blood from his brother stops the bleeding. (if he will have Anb agains platelets, his brother blood will just dilute his own blood,not eliminate the Abs, so would not stop the bleeding)
THE ONLY ONE LEFT IS 4!
Please let me know what is right! Thnaks

[ArchivalUser]

Had a similar Q in Q-Book & blundered there...
Cryopr. not correcting rules out Clottin factors,
Since platelet count is N. & it's got sumthing 2 do wid dysfunction leaving 3,4,5.
If antibodies is a problem, corrcted by 'brothers' platelets it shud a 55555!!!

[ArchivalUser]

Its Glycoprotein Ib deficiency i.e Bernard Souliers
also known as vWb factor RECEPTOR deficiency or MacroPlatelet disease.
It is very peculiar about it that a fresh transfusion of blood controls bleeding.
And you are right a similar Q is given in Kaplan Q book but I slightly modified it to make it two step that you should know both DDx and receptor before answering it......a UW style!

Glanzmans is Glycoprotein IIa/IIIb def

[ArchivalUser]

heyy...y not glanzman's....how would u diff it from bernard-soulier

[ArchivalUser]

Bernard-Soulier syndrome is a rare congenital bleeding disorder characterized by thrombocytopenia and large platelets.The underlying biochemical defect is the absent or decreased expression of the glycoprotein Ib/IX/V complex on the surface of the platelets. This complex is the receptor for von Willebrand factor

Glanzmann's thrombasthenia is an extremely rare disorder of the blood, in which the platelets lack glycoprotein IIb/IIIa. Hence, no fibrinogen bridging can occur, and bleeding time is significantly prolonged

[ArchivalUser]

As always....the first answer was correct answer.
Good one dude...!

[ArchivalUser]

An intelligent Q darkhorse!
any one wants to answer?
This is challenging DDx in this case.
Firstly there is no info given in the Q's which indicates presence of Autoantibodies to Glycoprotein.Secondly no info regarding 'Aggregation' of platelets which occurs in response to ristocetin, but not to other agonists such as ADP, thrombin, collagen or adrenalin.
And moreover,it is very important that there be a rigorous selection of
appropriate platelets starting with the first transfusions. This will help
delay the body™s natural defense system from rejecting platelet
transfusions that it sees as foreign.So an instant transfusion cured the patient slighly provokes mind to thin in other direction.
And lastly the Glanzman is one of the rarest diseases......In USMLE its the rule if you have doubt between two closely lying answer pick up the commom one.
Hope this will serve purpose.
NICE TO SEE YOUR PROMPT AND WISE DIFFRENTIAL

[ArchivalUser]

And it was the catch.........
Nice job drplacebo,
you picked up the correct answer earliest