0
A 2 2 -year-old male patien t presents with recu rrent epistaxis and a history of pro-longed bleeding after m i nor cuts. At age LJ, he had a splenectomy due to presumed immune th rombocytopenia. He is referred to hematology for evaluation of thrombo-cytopenia. His platelet count is LJ7 .000/ JLL. Hemoglobin and leukocyte coun ts are normal. A peripheral blood smear is shown below.
What test can be used as a confirmatory laboratory finding in the evaluation of Bernard-Soulier syndrome?
- Deficient ristocetin-dependent platelet agglutination
- Deficient primary and secondary response to ADP
- Normal agglutination studies
- Deficient collagen-dependent platelet agglutination
Medicine
A., Patients with BSS fail to aggregate with ristocetin and thus deficient ristocetin-dependent platelet aggregation is an indirect way to assess GPib-IX-V /VWF interaction, which is the underlying defect in Bernard-Soulier syndrome (Haematologica. 20 11;96:355-359).
need an explanation for this answer? contact us directly to get an explanation for this answer