Qualitative Platelet Disorders Hematology Review 343
DISORDER EXPLANATION LABORATORY TESTS
Inherited
Bernard-Soulier syndrome
Glanzmann’s thrombasthenia
d-storage pool disorder
Acquired
All can result in serious bleeding.
VWF = Von Willebrand factor.
Lack of functional glycoprotein (GP) Ib/IX/V on
plt surface prevents interaction with VWF.
Abnormal plt adhesion to collagen.
Deficiency or abnormality of plt membrane GP
IIb/IIIa. Fibrinogen can’t attach to plt surface &
initiate plt aggregation.
Dense granule deficiency. Lack of ADP release.
Functional plt disorders occur with chronic
renal failure, myeloproliferative disorders, car-
diopulmonary bypass, use of aspirin & other
drugs. Mechanisms vary.
Giant plts with dense granulation. ↑closure
time (PFA). Abnormal aggregation with
ristocetin.
↑closure time (PFA). Abnormal aggregation
with ADP, collagen, epinephrine.
Abnormal secondary aggregation with ADP &
epinephrine.
Abnormal plt aggregation.