ORIGINAL ARTICLESBleeding symptoms in heterozygous carriers of inherited coagulation disorders in southern IranMahmoodi, Mojtabaa,b; Peyvandi, Florac; Afrasiabi, AbdolRezaa; Ghaffarpasand, Fariborzd; Karimi, MehranaAuthor Information aHematology Research Center, Shiraz University of Medical Sciences, Shiraz bFasa University of Medical Sciences, Fasa, Iran cAngelo Bianchi Bonomi Hemophilia and Thrombosis Center; University of Milan, Milan, Italy dTrauma Research Center, Rajaee Hospital, Shiraz University of Medical Sciences, Shiraz, Iran Correspondence to Mehran Karimi, MD, Professor of Pediatrics Hematology Oncology, Hematology Research Center, Nemazee Hospital, Shiraz University of Medical Sciences, Shiraz, IranTel: +98 7116473239; fax: +98 7116474298; e-mail: email@example.com Received 20 October, 2010 Revised 5 February, 2011 Accepted 19 February, 2011 Blood Coagulation & Fibrinolysis: July 2011 - Volume 22 - Issue 5 - p 396-401 doi: 10.1097/MBC.0b013e328345f566 Buy Metrics Abstract The objective of the present study was to investigate the prevalence of bleeding symptoms in individuals who are heterozygous for recessively inherited coagulation disorders (RICDs) and to determine the association of these bleeding symptoms with type of RICDs. This was a retrospective cross-sectional study being performed in Shiraz Hemophilia Society (Shiraz, Southern Iran). In this study, bleeding symptoms of the parents (heterozygous) of the patients (homozygous) who were registered and had definite diagnosis as autosomal recessive coagulation disorder were evaluated. These inherited disorders include factor I, V, VII, X, XI, XIII deficiency, combined factor VII and X deficiency, combined factor V and VIII deficiency, all platelet disorders and von Willebrand disease (VWD) type III. 50.3% individuals underwent genotype and mutation study to confirm their heterozygosity. We included 350 heterozygote individuals for inherited coagulation disorders among whom there were 175 (50%) men and 175 (50%) women. Those who were heterozygous for factor VII deficiency had significantly higher prevalence of subcutaneous hematoma (P = 0.011). In the same way heterozygous patients for Bernard-Soulier syndrome had higher prevalence of hypermenorrhea (P = 0.012) and obstetric (normal vaginal delivery or cesarean delivery) bleeding (P = 0.012). Heterozygosity for factor X and XIII deficiency was associated with prolonged or massive bleeding during operations (P = 0.001) and after minor traumas (P = 0.019), respectively. Heterozygosity for RICDs is associated with some bleeding symptoms. Thus bleeding tendency and homeostasis disturbance should be kept in mind in those who are heterozygous for RICDs and more preoperative care and correction of coagulation indices is highly recommended. © 2011 Lippincott Williams & Wilkins, Inc.