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Platelets from "giant platelet syndrome (BSS)" are discocytes and normal sized
The Journal of Laboratory and Clinical Medicine
|January 1, 1978
Summary
Giant platelet size in Bernard-Soulier syndrome (BSS) appears during blood smear preparation, not in circulation. This suggests a defect in platelet shape change mechanisms, not inherent giantism.
Area of Science:
- Hematology
- Cell Biology
- Platelet Physiology
Background:
- Bernard-Soulier syndrome (BSS) is a rare bleeding disorder characterized by abnormally large platelets (giant platelets).
- The precise origin of giant platelet size in BSS has been a subject of investigation.
Purpose of the Study:
- To compare the shape and size of human platelets from normal donors and BSS donors.
- To investigate the influence of preparation methods on platelet morphology in BSS.
Main Methods:
- Cinematographic analysis of unfixed and glutaraldehyde-hardened platelets from citrated platelet-rich plasma (PRP).
- Evaluation of platelet morphology on blood smears from nine normal donors and two BSS donors.
- Measurement of platelet diameters and assessment of shape (discocyte, echinocyte).
Main Results:
- On blood smears, BSS platelets were 1.7-1.8 times larger than normal platelets, with many exceeding 2.5 micrometers.
- In PRP, 80-90% of BSS platelets were discocytes, indistinguishable from normal platelets.
- BSS echinocytes (spherical forms with pseudopods) had larger main body diameters and fewer pseudopods than normal.
Conclusions:
- The giant size of BSS platelets is an artifact of blood smear preparation, not an intrinsic property in circulation.
- Abnormal platelet shape changes during smear preparation likely contribute to the observed giantism.
- A defect in the platelet shape change mechanism is suggested as the underlying cause in BSS.