Patterns of platelet aggregation in selected disorders of platelet function and VWD

Disorder	Aggregation response				Other features
Disorder	Primary ADP	Secondary ADP	Collagen	Ristocetin	Other features
VWD	++++	++++	++++	Highly variable	Platelet morphology normal; VWD panel is usually abnormal
Bernard-Soulier syndrome	++++	++++	++++	0	Giant platelets, thrombocytopenia; VWD panel is normal
Glanzmann thrombasthenia	0	0	0	+++	Normal platelet morphology
Storage pool disease	++++	0 to ++	++	++	Platelet morphology normal (except in gray platelet subgroup); electron microscopy is abnormal
Secretion defect	++++	0 to ++	++	++	Normal morphology by light and electron microscopy

Expected aggregation responses in various disorders are illustrated. Refer to UpToDate for details. For inherited COX-1 defects or acquired interference with COX-1 function (eg, aspirin), testing can be done using arachidonic acid as the agonist. Results will show absent aggregation response to arachidonic acid as well as reduced secondary aggregation to ADP. Thromboxane receptor defects can be tested for using U-46619 in conjunction with this.

VWD: von Willebrand disease; ADP: adenosine diphosphate; COX-1: cyclooxygenase 1; ++++: normal response; +++: slightly reduced response; ++: reduced response; +: markedly reduced response; 0: no response.

Modified with permission from: Rodgers, GM. Qualitative platelet disorders and von Willebrand's disease. In: Practical Diagnosis of Hematologic Disorders, 2nd ed., Kjeldsberg, C, Foucar, K, McKenna, RW, et al. (Eds), ASCP Press, Chicago 1995. Copyright © 1995-2010 American Society for Clinical Pathology and ASCP Press.

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Patterns of platelet aggregation in selected disorders of platelet function and VWD