Previous reports have suggested that platelets from patients with Bernard-Soulier's syndrome (BSS) are not giant cells. Rather, they are normal-sized in suspension, but spread out on glass slides more readily than control cells, yielding the impression of being giant. The present study has used cell sizing techniques, electron microscopy, and micropipette aspiration to evaluate platelets from three patients with BSS. Cell sizing techniques revealed that BSS platelets were considerably larger than normal. The increased size was confirmed in electron microscopic studies of BSS platelets fixed in suspension. However, the BSS platelets did not contain increased amounts of internalized surface membrane considered to be the source of membrane necessary for excessive spreading. A possible explanation for increased spreading of BSS platelets was found in studies of their resistance to deformation in micropipettes. BSS platelets were much less resistant to deformation than normal cells or other abnormal platelets when aspirated under the same negative pressure. Their unusual deformability may explain the tendency of BSS platelets to spread more readily than normal cells on glass slides.
Micropipette aspiration of human blood platelets: a defect in Bernard- Soulier's syndrome
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JG White, SM Burris, D Hasegawa, M Johnson; Micropipette aspiration of human blood platelets: a defect in Bernard- Soulier's syndrome. Blood 1984; 63 (5): 1249–1252. doi: https://doi.org/10.1182/blood.V63.5.1249.bloodjournal6351249
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