Glanzmann's thrombasthenia

3,150views

Glanzmann's thrombasthenia

Blood and lymphoreticular system

Traumatic, mechanical, and vascular disorders

Flashcards

Glanzmann's thrombasthenia

0 of 10 complete

Questions

USMLE® Step 1 style questions USMLE

0 of 2 complete

A 9-year-old boy is brought to the emergency department by his parents due to prolonged bleeding following a tooth extraction earlier in the day. Past medical history is noncontributory. Temperature is 37.5°C (99.5°F), pulse is 88/min, respirations are 14/min, and blood pressure is 112/62 mmHg. Physical exam shows gingival bleeding and petechiae. Laboratory testing is obtained, and the results are shown below.  
 
Laboratory value  Result
 Hematologic  
 Hemoglobin  12 g/dL 
 Hematocrit  40% 
 Platelet count  95,000/mm3  
 Leukocyte count  9,000/mm3  
Coagulation studies  
 Prothrombin time (PT)  12 seconds 
 Activated partial thromboplastin time (aPTT)  29 seconds 
 Bleeding time 15 minutes 
*Reference Range: 2-7 minutes  

Which of the following conditions is the patient at greatest risk of developing?   

Key Takeaways

Glanzmann's thrombasthenia is a rare autosomal recessive bleeding disorder caused by defects in platelet aggregation. In Glanzmann's thrombasthenia, platelets are deficient in the fibrinogen receptor GpIIb/IIIa necessary for proper platelet aggregation. This leads to easy bruising, mucosa bleeding, and prolonged bleeding from even minor cuts. It can also cause internal bleeding, which can be life-threatening.