Biological Diagnosis and Physiopathology of von Willebrand-s Disease in a Part of the Algerian Population in the East and the South

Authors: H. Djaara, M. Yahia, H. Bousselsela, N Khelif, A. Zidani, S. Benbia.

Abstract:

Von Willebrand-s disease is the most common inherited bleeding disorder in humans, it caused by qualitative abnormalities of the von Willebrand factor (vWF). Our objective is to determine the prevalence of this disease at part of the Algerian population in the East and the South by a biological diagnosis based on specific biological tests (automated platelet count, the bleeding time (TS), the time of cephalin + activator (TCA), measure of the prothrombin rate (TP), vWF rate and factor VIII rate, Molecular electrophoresis of vWF multimers in agarose gel in the presence of SDS). Four patients of type III or severe Willebrand-s disease were found on 200 suspect cases. All cases are showed a deficit in vWF rate (< 5%), and factor VIII (P<0, 0001), and lengthening very significantly high of the TCA (P<0, 0001) and of the bleeding time (P<0,0001), with a normal blood platelet rate (P=0,7433) and a normal prothrombin rate (P=0,5808), an absence of all the multimers of vWF in plasma patients. The severe Willebrand-s disease is not only one pathology of primary haemostasis, but it can be accompanied by coagulation-s anomaly due to deficit in factor VIII. At this studied population, von Willebrand-s disease is less frequent (2%) than other hemorrhagic syndromes identified by the differential diagnosis like the thrombocytopenia (36%).

Keywords: Von Willebrand's disease, differential diagnosis, von Willebrand factor, factor VIII, biological diagnosis, thrombocytopenia.

Digital Object Identifier (DOI): doi.org/10.5281/zenodo.1070409

References:

[1] T. De Revel., K. Doghmi, "The Normal Haemostatic Process", EMCDentisteriy, pp. 1:71-81, 2004.
[2] E. Fressinaud, D.Meyer, "Von Willebrand-s disease, Medico-Surgical Encyclopaedia", pp. 13-021-A-50, 2001.
[3] A. Borel Derlon, "Haermostasis drugs in urgency perioperative", Fr Ann of Anesth Reanim, pp. 17 (Suppel 1): 10-4, 1998.
[4] S.Cherkaoui, A. Laaloui, S. Faiz, N.Benchemsi, "von Willebrand-s disease and delivery in a patient with Willebrand-s disease", Clinical and Biological transfusion, pp.14: 474-480, 2007.
[5] C. Pommier, C. Perrin, R. Dorne, and al.,"Haemoperitoeum and pregnancy in a patient with von Willebrand-s disease type 3", Fr Ann of Anesth Reanim, pp. 21: 436-9, 2002.
[6] C. Rothschild, "von Willebrand-s disease", Clin Biol Transfus, Elsevier (Paris), pp. 5: 357-61, 1998.
[7] SW. Davies, B. Marchant, L.yons, "Irregular coronary lesion morphology after thrombolyis predicts early clinical instability", J Am Coll Cardiol, pp. 18: 669-74, 1991.
[8] R. Zittoun, M. Samama, JP. Mairie, "Manual of hematology", p. 446, 1993.
[9] DD. Wagner, "Cell biology of von Willebrand factor", Annu Rev Cell Biol, pp. 6: 217-46, 1990.
[10] Sadler JE. "Biochemistry and genetics of von Willebrand factor", Annu Rev Biochem pp. 67: 395-424, 1998.
[11] M.C. Trzeciak, JC. Bordet, "Exploration of primary haemostasis", Med Surg Encycl Scientific Editions and Medicals Elsevier SAS, Paris, Hematology, 5 pp. 13-0 19-A-10, 2002.
[12] E. Joseph, "Blood vessels-s disease", p. 379, 1998.
[13] RW. Colman, AW. Clowes, JN. George, SZ. Goldhaber, VJ. Marder, eds. Overview of hemostasis, In: "Hemostasis and Thrombosis: Basic Principles and Clinical Practice", 5th ed. Philadelphia, Pa: JB Lippincott Co; pp. 3-16. 2006.
[14] C. Hermans, B. Dessomme, C. Lambert, and al., "Venous malformations and coagulopathy", Annals of plastic and Aesthetic surgery, pp. 51: 388-393, 2006.
[15] JP. Lévy, B.Varet, JP. Clauvel, and al. "Hematology and transfusion", p. 384, 2001.
[16] R. Schneppenheim, S. Krey, F. Bergmann, D. Bock, U. Budde, M. Lange, R. Linde, U.Mittler, E. Meili, G. Mertes, K. Olek, H. Plendl, E. Simeoni, "Genetic heterogeneity of severe von Willebrand disease type III in the German population", Hum Genet, pp. 94: 640-52,1994
[17] L L. Géraldine, D. Elodie, B. Sophie, "The thrombocytopenias: State of place 2005". 8pp 26-33, 2006.