Platelets Failed to Aggregate! - USMLE Forums
USMLE Forums Logo
USMLE Forums         Your Reliable USMLE Online Community     Members     Posts
Home
USMLE Articles
USMLE News
USMLE Polls
USMLE Books
USMLE Apps
Go Back   USMLE Forums > USMLE Step 2 CK Forum

USMLE Step 2 CK Forum USMLE Step 2 CK Discussion Forum: Let's talk about anything related to USMLE Step 2 CK exam


Reply
 
Thread Tools Search this Thread Display Modes
  #1  
Old 09-09-2012
K06100's Avatar
USMLE Forums Master
 
Steps History: 1+CK+CS+3
Posts: 730
Threads: 90
Thanked 458 Times in 289 Posts
Reputation: 468
Blood Platelets Failed to Aggregate!

A one and half-year-old girl is sent to a children's hospital for evaluation following a nosebleed which was so severe as to require nasal packing and transfusion of platelet concentrates. When a blood sample had been drawn in the emergency room for serum chemistry studies, the local hospital laboratory had noted that the clot that formed was unusual in that it failed to retract. Peripheral blood smear obtained by finger puncture showed an appropriate number of normal-sized platelets, all of which were individual, without clumping. At the children's hospital, it was noted that the child's parents were cousins. Special platelet studies showed that the child's platelet's failed to aggregate with any physiologic aggregating agent, including a high concentration of exogenous ADP. Which of the following is the most likely diagnosis?

a) Bernard-Soulier syndrome
b) Chediak-Higashi syndrome
c) May-Hegglin anomaly
d) Thrombasthenia
e) Von Willebrand disease
__________________
Try Not To Become A Man Of Success But A Man Of Value.
Reply With Quote Quick reply to this message
The above post was thanked by:
Novobiocin (09-09-2012), step1an (09-09-2012)



  #2  
Old 09-09-2012
USMLE Forums Master
 
Steps History: 1+CK+CS+3
Posts: 791
Threads: 76
Thanked 673 Times in 317 Posts
Reputation: 691
Default

D....glanzmann thrombesthenia....GP 2b/3a def
Reply With Quote Quick reply to this message
The above post was thanked by:
K06100 (09-09-2012)
  #3  
Old 09-09-2012
USMLE Forums Guru
 
Steps History: 1 + CK
Posts: 373
Threads: 30
Thanked 375 Times in 160 Posts
Reputation: 385
Default

Yes .... D
__________________
Knowing is not enough; we must apply.
Willing is not enough; we must do.
Reply With Quote Quick reply to this message
The above post was thanked by:
K06100 (09-09-2012)
 
  #4  
Old 09-09-2012
USMLE Forums Master
 
Steps History: 1+CK+CS
Posts: 720
Threads: 217
Thanked 552 Times in 309 Posts
Reputation: 562
Default

Yes Platlet function defect 2b3a def - Glanzmans thrombasthenia..

D
Reply With Quote Quick reply to this message
The above post was thanked by:
K06100 (09-09-2012)
  #5  
Old 09-09-2012
XpaezX's Avatar
USMLE Forums Master
 
Steps History: 1+CK+CS+3
Posts: 1,216
Threads: 41
Thanked 1,327 Times in 711 Posts
Reputation: 1342
Default

Glanzmann thromboasthenia

Clue is that platelets suck but are NORMAL SIZED

In Bernard Soulier they are big and weird, also the problem is with Gp1 receptor
Reply With Quote Quick reply to this message
The above post was thanked by:
K06100 (09-09-2012)
  #6  
Old 09-09-2012
USMLE Forums Master
 
Steps History: 1+CK+CS
Posts: 1,867
Threads: 149
Thanked 2,106 Times in 1,049 Posts
Reputation: 2126
Default

d)Thrombasthenia

Quote:
Glanzmann's thrombasthenia is an abnormality of platelets.[1] It is an extremely rare coagulopathy (bleeding disorder due to a blood abnormality), in which the platelets lack glycoprotein IIb/IIIa (GpIIb/IIIa). As a result, no fibrinogen bridging of platelets to other platelets can occur, and the bleeding time is significantly prolonged.
Platelet numbers and morphology are normal. Platelet aggregation is normal with ristocetin, but impaired with other agonists such as ADP, thrombin, collagen or epinephrine.
Quote:
Bernard–Soulier syndrome (BSS), also called hemorrhagiparous thrombocytic dystrophy,[1] is a rare autosomal recessive coagulopathy (bleeding disorder) that causes a deficiency of glycoprotein Ib (GpIb), the receptor for von Willebrand factor, which is important in clot formation.
The incidence is estimated to be less than 1 in 1 million persons, based on cases reported from Europe, North America, and Japan.[2]
It is a Giant Platelet Syndrome that is characterized by abnormally large platelets.
BSS platelets do not aggregate to ristocetin, and this defect is not corrected by the addition of normal plasma, distinguishing it from von Willebrand disease. The platelet responses to physiologic agonists is normal, with the exception of low concentrations of thrombin. Bleeding events, which may be very severe, can be controlled by platelet transfusion. Most heterozygotes, with few exceptions, do not have a bleeding diathesis. It presents as a bleeding disorder due to the inability of platelets to bind and aggregate at sites of vascular endothelial injury.[3]

Last edited by Novobiocin; 09-09-2012 at 03:49 PM.
Reply With Quote Quick reply to this message
The above post was thanked by:
K06100 (09-10-2012), step1an (09-09-2012)



Reply

Tags
Hematology-, Pediatrics-, Step-2-Questions

Quick Reply
Message:
Options

Register Now

In order to be able to post messages on the USMLE Forums forums, you must first register.
Please enter your desired user name, your email address and other required details in the form below.
User Name:
Password
Please enter a password for your user account. Note that passwords are case-sensitive.
Password:
Confirm Password:
Email Address
Please enter a valid email address for yourself.
Email Address:
Medical School
Choose "---" if you don't want to tell. AMG for US & Canadian medical schools. IMG for all other medical schools.
USMLE Steps History
What steps finished! Example: 1+CK+CS+3 = Passed Step 1, Step 2 CK, Step 2 CS, and Step 3.

Choose "---" if you don't want to tell.

Favorite USMLE Books
What USMLE books you really think are useful. Leave blank if you don't want to tell.
Location
Where you live. Leave blank if you don't want to tell.

Log-in

Human Verification

In order to verify that you are a human and not a spam bot, please enter the answer into the following box below based on the instructions contained in the graphic.



Thread Tools Search this Thread
Search this Thread:

Advanced Search
Display Modes


Similar Threads
Thread Thread Starter Forum Replies Last Post
failed nushah USMLE Step 1 Forum 1 08-19-2012 05:42 PM
Failed with 192 oapi USMLE Step 2 CK Forum 15 08-15-2012 05:41 PM
failed cs rsin21 USMLE Step 2 CS Forum 2 06-22-2012 04:11 PM
Etiology of hyperkalemia; increased platelets and WBC count! Dr. N Elham USMLE Step 2 CK Forum 2 06-05-2011 06:40 PM

RSS Feed
Find Us on Facebook
vBulletin Security provided by vBSecurity v2.2.2 (Pro) - vBulletin Mods & Addons Copyright © 2017 DragonByte Technologies Ltd.

USMLE® & other trade marks belong to their respective owners, read full disclaimer
USMLE Forums created under Creative Commons 3.0 License. (2009-2014)