The Hermansky-Pudlak Syndrome (HPS) is an autosomal recessive inherited disorder characterized by oculocutaneous albinism, tissue accumulation of ceroid pigment, and a mild to moderate bleeding diathesis attributed to storage-pool deficient (SPD) platlets. Patients have platelet aggregation and rele
β¦ LIBER β¦
Platelet von willebrand factor abnormalities in myeloproliferative syndromes
β Scribed by Dr. Giancarlo Castaman; Antonella Lattuada; Marco Ruggeri; Alberto Tosetto; Pier Mannuccio Mannucci; Francesco Rodeghiero
- Publisher
- John Wiley and Sons
- Year
- 1995
- Tongue
- English
- Weight
- 496 KB
- Volume
- 49
- Category
- Article
- ISSN
- 0361-8609
No coin nor oath required. For personal study only.
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## Abstract The most common abnormality of the platelets in 43 patients with a variety of myeloproliferative syndromes was impaired or absent aggregation when stimulated with collagen and adrenaline. Eight unselected cases studied in more detail showed a normal prostaglandin synthesis pathway as ev
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