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
- 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
- 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
- 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
- 5 Central Intelligence Agency .The World Factbook: Iran. Available at: https://www.cia.gov/library/publications/the-world-factbook/geos/ir.html
- 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
- 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
- 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
- 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
- 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
- 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
- 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
- 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
- 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
- 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
- 16
Mannucci P M.
Hemostatic drugs.
N Engl J Med.
1998;
339
(4)
245-253
- 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
Mehran KarimiM.D.
Professor of Pediatric Hematology-Oncology, Hematology Research Center
Nemazee Hospital, Shiraz, Iran
Email: karimim@sums.ac.ir
