Biological Diagnosis and Physiopathology of von Willebrand-s Disease in a Part of the Algerian Population in the East and the South
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%).
[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.
[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.
@article{"International Journal of Medical, Medicine and Health Sciences:57261", author = "H. Djaara and M. Yahia and H. Bousselsela and N Khelif and A. Zidani and S. Benbia.", title = "Biological Diagnosis and Physiopathology of von Willebrand-s Disease in a Part of the Algerian Population in the East and the South", 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", keywords = "Von Willebrand's disease, differential diagnosis, von
Willebrand factor, factor VIII, biological diagnosis,
thrombocytopenia.", volume = "6", number = "3", pages = "51-4", }