Morphometric Analysis of Platelets in Bernard-Soulier Syndrome: Size and Configuration in Patients and Carriers

Summary

Quantitative ultrastructural morphometric analysis has been carried out on thin sections of platelets from two Bernard-Soulier sisters and their parents. Measurements were made for the major and minor axes, axial ratios, cross-sectional circumference and cross-sectional area. Platelets were collected either into CPD anticoagulant or directly into glutaraldehyde. The results confirm that Bemard-Soulier platelets are significantly larger than normals (p <0.05) in all the parameters mentioned and indicate (I) that the morphology of platelets from Bernard-Soulier patients is affected by the presence of anticoagulant, and (II) a more spherocytic configuration is characteristic of Bemard-Soulier disease both in patients and carriers.

Keywords

Bemard-Soulier - Ultrastructure - Morphometric analysis - Platelets

• References

• 1 Howard MS, Hutton RA, Hardisty RM. Hereditary giant platelet syndrome: a disorder of a new aspect of platelet function. Br Med J 1973; 2: 586-588
• 2 Jamieson GA, Okumura T. Reduced thrombin binding and aggregation in Bernard-Soulier platelets. J Clin Invest 1978; 61: 861-864
• 3 Weiss JH, Tschopp TB, Baumgartner HR, Susmann II, Johnson MM, Egan JJ. Decreased adhesion of giant (Bernard-Soulier) platelets to subendothelium. Further implications on the role of von Willebrand factor in hemostasis Am J Med 1974; 57: 920-925
• 4 Grøttum KA, Solum NO. Congenital thrombocytopenia with giant platelets: A defect in the platelet membrane. Br J Haematol 1969; 16: 277-290
• 5 Nurden AT, Caen JP. Specific roles for surface glycoproteins in platelet function. Nature 1975; 255: 720-722
• 6 Jenkins CS P, Phillips DR, Clemetson KJ, Meyer D, Larrieu MJ, Lücher EF. Platelet membrane glycoproteins implicated in ristocetin induced aggregation. J Clin Invest 1976; 57: 112-114
• 7 Jamieson GA, Okumura T, Fishback B, Johnson MM, Egan JJ, Weiss JH. Platelet membrane glycoproteins in thromboasthenia, Bernard-Soulier Syndrome, and storage pool disease. J Lab Clin Med 1979; 93: 652-660
• 8 Bernard J, Soulier J-P. Sur une nouvelle varieté du dystrophie thrombocytaire hémorragipare congénitale. Sem Hop (Paris) 1948; 24: 3217-3223
• 9 Niewiaroski S, Poplowski A, Prokopowicsz J. Abnormalities of platelet function and ultrastructure in macrothrombocytic thrombo- cytopathia. Scand J Haematol 1969; 6: 377-385
• 10 Pavlovsky A, Tezanos D, Pinto M, Martinez CamaveriA. A propos d’un cas de thrombocytopathie hémorragique congénitale (et au microscopy électronique). Nouv Rev Fr Haematol 1976; 7: 559-564
• 11 Maldonado JE, Gilchrist GS, Brigden LP, Bowie EJ W. Ultrastructure of platelets in Bemard-Soulier syndrome. Mayo Clinic Proc 1975; 50: 402-406
• 12 Chevalier J, Nurden AT, Thiery JM, Savariau E, Caen JP. Freeze fracture studies on the plasma membranes of normal, thrombasthenic and Bemard-Soulier platelets. J Lab Clin Med 1979; 94: 232-245
• 13 Frojmovic MM, Milton JG, Caen JP, Tobelem G. Platelets from “giant platelets syndrome (BSS)” are discocytes and normal sized. J Lab Clin Med 1978; 91: 109-116
• 14 Milton JG, Frojmovic MM. Invaginated plasma membrane of human platelets: Evagination and measurement in normal and “giant platelets”. J Lab Clin Med 1979; 93: 162-170
• 15 Rock G, Ordinas A, Drouin J, Wong S-C, Jamieson GA. Glycoprotein I is not involved in aggregation of platelets by wheat germ agglutinin. Thromb Res 1980; 19: 725-728
• 16 Evensen SA, Solum MO, Grøttum KA, Hovig T. Familial bleeding disorder with a moderate thrombocytopenia and giant blood platelets. Scand J Hematol 1974; 13: 203-214
• 17 Winer BJ. Statistical Principles in Experimental Design. 2. edition McGraw-Hill: 1971. pp 149-157
• 18 Barr AJ, Goodnight JH, Sall JP, Blair WH, Chilko DM. SAS user’s guide. Helwig JT, Council KA. (eds) SAS Institute; Raleigh NC: 1979. pp 3-85 237-264 427-432
• 19 Rendu F, Nurden AT, LeBret M, Caen JP. Further investigations on Bemard-Soulier platelet abnormalities. A study of 5-hydroxytrypt- amine uptake and mepacrine fluorescence J Lab Clin Med 1981; 97: 689-699
• 20 Nurden AT, Dupuis D, Kunicki J, Caen JP. Analysis of the glycoprotein and protein composition of Bemard-Soulier platelets by single and two-dimensional sodium dodecyl sulfate-polyacrylamide gel electrophoresis. J Clin Invest 1981; 67: 1431-1440
• 21 Stahl K, Themann H, Dame WR. Ultrastructural morphometric investigation on normal human platelets. Hemostasis 1978; 7: 242-251
• 22 George JN, Reimann TA, Moake JL, Morgan RK, Cimo PL, Sears DA. Bemard-Soulier disease: a study of four patients and their parents. Br J Haematol 1981; 48: 459-467