The storage defects in grey platelet syndrome and αδ-storage pool deficiency affect α-granule factor V and multimerin storage without altering their proteolytic processing
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چکیده
منابع مشابه
Platelet storage pool deficiency in Jacobsen syndrome.
Jacobsen syndrome and Paris-Trousseau Syndrome share similar congenital anomalies, thrombocytopenia, giant platelet alpha granules resulting from fusion of smaller organelles, and an 11q terminal deletion at 11q23.3. Similarities in the two cohorts have suggested that the Paris-Trousseau Syndrome is a variant of Jacobsen syndrome, or the same disorder. The present study has pointed out a signif...
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We report a new bleeding disease--storage pool deficiency (SPD) of platelets--in pigs from the Mayo swine colony of homozygous von Willebrand's disease (vWD) and of heterozygous carriers of vWD. Levels of factor VIII, von Willebrand factor antigen (vWF:Ag), and ristocetin cofactor (RCof) were similar in the vWD carriers and SPD pigs. The latter pigs, however, had bleeding times of 15 minutes or...
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چکیده ندارد.
15 صفحه اولSerum antibody-negative Goodpasture syndrome with delta granule pool storage deficiency and eosinophilia
Goodpasture syndrome is a rare, life-threatening autoimmune disease characterized by a triad of rapidly progressive glomerulonephritis, a hemorrhagic pulmonary condition and the presence of anti-glomerular basement membrane (anti-GBM) antibodies. The antibodies initiate destruction of the kidney glomeruli, resulting in a focal necrotizing glomerulitis, which may progress rapidly to renal failur...
متن کاملPostural orthostatic tachycardia syndrome is associated with platelet storage pool deficiency
Mechanisms have been postulated to explain postural orthostatic tachycardia syndrome (POTS), however, the etiology of this often debilitating disorder remains unknown. We conducted a retrospective case-control study of 181 POTS patients who exhibited/reported bleeding symptoms for a specific platelet (PL) dysfunction disorder, delta granule storage pool deficiency (δ-SPD).Patients were included...
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ژورنال
عنوان ژورنال: British Journal of Haematology
سال: 2001
ISSN: 0007-1048
DOI: 10.1046/j.1365-2141.2001.02833.x