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Bleeding Disorder due to an Isolated Platelet Factor 3 Deficiency

I. Monica Minkoff, MD; Kenneth K. Wu, MD; Joan Walasek; Bernie Lightfoot; Cassandra Smith-McKearn
Arch Intern Med. 1980;140(3):366-367. doi:10.1001/archinte.1980.00330150080020.
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• To determine the clinical and functional characteristics of isolated platelet factor 3 (PF-3) deficiency, platelet function studies were carried out in 316 patients who were suspected of having a bleeding tendency. Although PF-3 abnormality was observed in 69 patients (22%), an isolated PF-3 abnormality was noted in only three patients. The abnormality was congenital in one patient and secondary to Hashimoto's thyroiditis, Ehlers-Danlos syndrome, and chronic myelocytic leukemia in the other two. Further investigations in the patient with congenital abnormality disclosed that PF-3 activity failed to respond to any of the stimuli tested, and there was a clear dissociation between PF-3 availability and platelet aggregation. The abnormality was due to an intrinsic platelet defect caused by a true deficiency of the membrane phospholipid clotting activity. The findings clearly establish that isolated PF-3 deficiency is an entity of qualitative platelet disorders that may be associated with a severe bleeding tendency.

(Arch Intern Med 140:366-367, 1980)


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