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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.;

Biological Diagnosis And Physiopathology Of Von Willebrand-S Disease In A Part Of The Algerian Population In The East And The South

Abstract

{"references": ["T. De Revel., K. Doghmi, \"The Normal Haemostatic Process\", EMCDentisteriy,\npp. 1:71-81, 2004.", "E. Fressinaud, D.Meyer, \"Von Willebrand-s disease, Medico-Surgical\nEncyclopaedia\", pp. 13-021-A-50, 2001.", "A. Borel Derlon, \"Haermostasis drugs in urgency perioperative\", Fr Ann\nof Anesth Reanim, pp. 17 (Suppel 1): 10-4, 1998.", "S.Cherkaoui, A. Laaloui, S. Faiz, N.Benchemsi, \"von Willebrand-s\ndisease and delivery in a patient with Willebrand-s disease\", Clinical and\nBiological transfusion, pp.14: 474-480, 2007.", "C. Pommier, C. Perrin, R. Dorne, and al.,\"Haemoperitoeum and\npregnancy in a patient with von Willebrand-s disease type 3\", Fr Ann of\nAnesth Reanim, pp. 21: 436-9, 2002.", "C. Rothschild, \"von Willebrand-s disease\", Clin Biol Transfus, Elsevier\n(Paris), pp. 5: 357-61, 1998.", "SW. Davies, B. Marchant, L.yons, \"Irregular coronary lesion\nmorphology after thrombolyis predicts early clinical instability\", J Am\nColl Cardiol, pp. 18: 669-74, 1991.", "R. Zittoun, M. Samama, JP. Mairie, \"Manual of hematology\", p. 446,\n1993.", "DD. Wagner, \"Cell biology of von Willebrand factor\", Annu Rev Cell\nBiol, pp. 6: 217-46, 1990.\n[10] Sadler JE. \"Biochemistry and genetics of von Willebrand factor\", Annu\nRev Biochem pp. 67: 395-424, 1998.\n[11] M.C. Trzeciak, JC. Bordet, \"Exploration of primary haemostasis\", Med\nSurg Encycl Scientific Editions and Medicals Elsevier SAS, Paris,\nHematology, 5 pp. 13-0 19-A-10, 2002.\n[12] E. Joseph, \"Blood vessels-s disease\", p. 379, 1998.\n[13] RW. Colman, AW. Clowes, JN. George, SZ. Goldhaber, VJ. Marder,\neds. Overview of hemostasis, In: \"Hemostasis and Thrombosis: Basic\nPrinciples and Clinical Practice\", 5th ed. Philadelphia, Pa: JB Lippincott\nCo; pp. 3-16. 2006.\n[14] C. Hermans, B. Dessomme, C. Lambert, and al., \"Venous\nmalformations and coagulopathy\", Annals of plastic and Aesthetic\nsurgery, pp. 51: 388-393, 2006.\n[15] JP. L\u00e9vy, B.Varet, JP. Clauvel, and al. \"Hematology and transfusion\", p.\n384, 2001.\n[16] R. Schneppenheim, S. Krey, F. Bergmann, D. Bock, U. Budde, M.\nLange, R. Linde, U.Mittler, E. Meili, G. Mertes, K. Olek, H. Plendl, E.\nSimeoni, \"Genetic heterogeneity of severe von Willebrand disease type\nIII in the German population\", Hum Genet, pp. 94: 640-52,1994\n[17] L L. G\u00e9raldine, D. Elodie, B. Sophie, \"The thrombocytopenias: State of\nplace 2005\". 8pp 26-33, 2006."]}

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

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

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