Subscribe to RSS
DOI: 10.1055/s-0031-1281049
Diagnosis and Management of von Willebrand Disease in Iran
Publication History
Publication Date:
18 November 2011 (online)

ABSTRACT
Little information is available on von Willebrand disease (VWD) in Iran. More than 900 patients with VWD are registered, but the real number of patients with VWD is likely to be larger. Fifteen hemophilia treatment centers in addition to 31 other academic centers are the main sites for the clinical management and treatment of VWD in Iran. All centers are staffed by specialists in the medical care of VWD. Epistaxis and menorrhagia are the most frequent clinical manifestations in Iranian patients with VWD. von Willebrand factor/factor VIII concentrates, desmopressin, cryoprecipitates, and tranexamic acid are the main medications used routinely in Iranian patients, and they are administered based on the type of disease, the severity of bleeding, and sometimes for the prevention of bleeding during surgical procedures or delivery. It is hoped that the recent creation of a national registry for coagulation disorders and improvements in laboratory techniques will improve the diagnosis, national patients' registry, and management of VWD in Iranian patients and lead to gains in their quality of life in the near future.
KEYWORDS
von Willebrand disease - diagnosis - management - Iran
REFERENCES
- 1
Mannucci P M.
Treatment of von Willebrand's disease.
N Engl J Med.
2004;
351
(7)
683-694
MissingFormLabel
- 2
Sadler J E, Budde U, Eikenboom J C Working Party on von Willebrand Disease Classification et al.
Update on the pathophysiology and classification of von Willebrand disease: a report of the Subcommittee on von Willebrand factor.
J Thromb Haemost.
2006;
4
(10)
2103-2114
MissingFormLabel
- 3
Dilley A, Drews C, Miller C et al..
von Willebrand disease and other inherited bleeding disorders in women with diagnosed
menorrhagia.
Obstet Gynecol.
2001;
97
(4)
630-636
MissingFormLabel
- 4
Shoa'i I, Lavergne J M, Ardaillou N, Obert B, Ala F, Meyer D.
Heterogeneity of von Willebrand's disease: study of 40 Iranian cases.
Br J Haematol.
1977;
37
(1)
67-83
MissingFormLabel
- 5 Central Intelligence Agency .The World Factbook: Iran. Available at: https://www.cia.gov/library/publications/the-world-factbook/geos/ir.html
MissingFormLabel
- 6
Lak M, Peyvandi F, Mannucci P M.
Clinical manifestations and complications of childbirth and replacement therapy in
385 Iranian patients with type 3 von Willebrand disease.
Br J Haematol.
2000;
111
(4)
1236-1239
MissingFormLabel
- 7
Gadisseur A, Hermans C, Berneman Z, Schroyens W, Deckmyn H, Michiels J J.
Laboratory diagnosis and molecular classification of von Willebrand disease.
Acta Haematol.
2009;
121
(2-3)
71-84
MissingFormLabel
- 8
Yawn B, Nichols W L, Rick M E.
Diagnosis and management of von Willebrand disease: guidelines for primary care.
Am Fam Physician.
2009;
80
(11)
1261-1268
MissingFormLabel
- 9
Shahbazi S, Mahdian R, Ala F A, Lavergne J M, Denis C V, Christophe O D.
Molecular characterization of Iranian patients with type 3 von Willebrand disease.
Haemophilia.
2009;
15
(5)
1058-1064
MissingFormLabel
- 10
Baronciani L, Cozzi G, Canciani M T et al..
Molecular defects in type 3 von Willebrand disease: updated results from 40 multiethnic patients.
Blood Cells Mol Dis.
2003;
30
(3)
264-270
MissingFormLabel
- 11 University of Sheffield; International Society on Thrombosis and Haemostasis .International Society on Thrombosis and Haemostasis Science Standardization Committee
von Willebrand Disease database. Available at: http://www.vwf.group.shef.ac.uk
MissingFormLabel
- 12
Gill J C, Ewenstein B M, Thompson A R, Mueller-Velten G, Schwartz B A. Humate-P Study Group .
Successful treatment of urgent bleeding in von Willebrand disease with factor VIII/VWF concentrate (Humate-P): use of the ristocetin
cofactor assay (VWF:RCo) to measure potency and to guide therapy.
Haemophilia.
2003;
9
(6)
688-695
MissingFormLabel
- 13
Karimi M, Ghavanini A A.
Seroprevalence of HBsAg, anti-HCV, and anti-HIV among haemophiliac patients in Shiraz,
Iran.
Haematologia (Budap).
2001;
31
(3)
251-255
MissingFormLabel
- 14
Federici A B, Mazurier C, Berntorp E et al..
Biologic response to desmopressin in patients with severe type 1 and type 2 von Willebrand disease: results of a multicenter European study.
Blood.
2004;
103
(6)
2032-2038
MissingFormLabel
- 15
Boda Z, Pfliegler G, Hársfalvi J, Rak K.
Treatment of the severe bleeding episode in type III von Willebrand's disease by simultaneous administration of cryoprecipitate and platelet
concentrate.
Blood Coagul Fibrinolysis.
1991;
2
(6)
775-777
MissingFormLabel
- 16
Mannucci P M.
Hemostatic drugs.
N Engl J Med.
1998;
339
(4)
245-253
MissingFormLabel
- 17
Kadir R A, Lee C A, Sabin C A, Pollard D, Economides D L.
Pregnancy in women with von Willebrand's disease or factor XI deficiency.
Br J Obstet Gynaecol.
1998;
105
(3)
314-321
MissingFormLabel
Mehran KarimiM.D.
Professor of Pediatric Hematology-Oncology, Hematology Research Center
Nemazee Hospital, Shiraz, Iran
Email: karimim@sums.ac.ir