The Evaluation of the Relationship between Plasma Levels of Factor VIII with Bleeding Symptoms in Hemophilia a Carriers Referring to Shafa Hospital of Ahvaz, Iran

Document Type : Original Article

Authors

1 Professor of Pediatrics, Research Committee, Ahvaz Jundishapur University of Medical Sciences, Ahvaz,

2 Medical Student, Faculty of Medicine, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran

Abstract

Background and Objective: Hemophilia is an X linked bleeding disorder due to the decreased level of coagulation factors. In this disease bleeding in different parts of the body manifested as bruising and bleeding in gastrointestinal, musculoskeletal, genitourinary and other organs. The aim of this study was to investigate the role of factor VIII as a cause factor for bleeding among hemophilia A carries.
Subjects and Methods: This study was based on a preliminary interview with carriers of hemophilia A to gather information on the occurrence and symptoms of the bleeding experienced along with family records. Then the serum level of factor VIII was measured. A total of 70 patients were selected for the study based on sample size. The data obtained were analyzed using SPSS software.
Results: Lack of factor VIII on its own can increase the risk of bleeding in patients with hemophilia A carriers. Based on the data analysis, factor VIII serum level less than and in the range of 40-45 carries an increased risk of bleeding. Bleeding from different sits such as gums, mouth, and menorrhagia alone are not dependent on the level of factor VIII, however, it is level is contributing factor in bleeding tendency
Discussion: Due to the increased risk of bleeding in patients with lower levels of factor VIII, it is necessary to fully scrutinize these patients by assessing serum level of factor VIII and in terms of the development of bleeding. In addition, in order to prevent further complications, increase in awareness among these patients of their condition can be helpful.
Key words: Hemophilia A, Bleeding, Factor VIII.

Keywords

References

 
1-Plug  I, Mauser-Bunschoten EP, Bröcker-Vriends AH, van Amstel HK, van der Bom JG, van Diemen-Homan JE, et al. Bleeding in carriers of hemophilia. Blood 2006; 108(1): 52-6.
2-Fatehifar  MR,  Horfar  H, Akbari  N, Eshraghi  M,  Hojatifar M, Motamedi  N, et al. The correlation of factor VIII and von Willebrand levels with clinical symptoms in obligate carriers of hemophilia A in Isfahan. Sci J Blood Transfus Organ 2014; 11(2): 110-115
3-Ay C, Thom K, Abu-Hamdeh F, Horvath B, Quehenberger P, Male C, et al. Determinants of factor VIII plasma levels in carriers of haemophilia A and in control women. Haemophilia 2010; 16(1): 111-7.
4-Kadir RA, Economides DL, Braithwaite J, Goldman E, Lee CA.The obstetric experience of carriers of haemophilia. Br JObstet Gynaecol 1997; 104(7): 803-10. 
5-Winikoff R, Lee C. Hemophilia carrier status and counseling the symptomatic and asymptomatic adolescent. J Pediatr Adolesc Gynecol 2010; 23(6): S43-7.
6-Merskey C. The occurrence of haemophilia in the human female. Q J Med 1951; 20(79): 299-312.
7-Mauser-Bunschoten EP. Symptomatic carriers of Hemophilia. World Federation of Hemophilia 2008; 46: 2-3.
8-Rodeghiero F, Tosetto A, Castaman G. How to estimate bleeding risk in mild bleeding disorders. J Thromb Haemost 2007; 5 Suppl 1: 157-66.
9-Street AM, Ljung RL, Lavery SA. Management of carriers and babies with haemophilia. Haemophilia 2008; 14 Suppl 3: 181-7.
10-Higham JM, O'Brien PM, Shaw RW. Assessment of menstrual blood loss using a pictorial chart. Br J Obstet Gynaecol 1990; 97(8): 734-9.
11-Merskey C. The occurrence of haemophilia in the human female. Q J Med 1951; 20(79): 299-312.
12-Klein HG, Aledort LM, Bouma BN, Hoyer LW,Zimmerman TS, DeMets DL. A co-operative study for the detection of the carrier state of classic hemophilia. N Engl J Med 1977; 296(17): 959-62.
13-Ratnoff OD, Jones PK. The laboratory diagnosis of the carrier state for classic hemophilia. Ann Intern Med 1977; 86(5): 521-8.
14-Alvarez-Leal M, Perez-Vazquez JC, Gaspar-Belmonte JA, Castillo-Solis T, Zuñiga-Charles MA. Carrier detection of hemophilia by immunological and coagulation assays in a selected sample of the Mexican population. Am J Hum Biol 1997; 9(2): 173-8.
15-Rodeghiero F, Castaman G, Tosetto A, Battle J, Baudo F, Cappelletti A, et al. The discriminant power of bleeding history for the diagnosis of von Willebrand disease type 1: an international,multicenter study. J Thromb Haemost 2005; 3(12): 2619-26.
16-Iris Plug, Eveline P. Mauser-Bunschoten, Annette H. J. T. Bröcker-Vriends, Hans Kristian Ploos van Amstel. Bleeding in carriers of hemophilia. Blood 2006 108:52-56
17-Grunewald M,Beneke H,Guthner C,Germowitz A,Brommer A Griesshammer M (2001) Acquired haemophilia: experiences with a standardized approach. Haemophilia 7:164–9.
18-Verbruggen B, Novakova I, Wessels H, Boezeman J, van den Berg M Mauser-Bunschoten E  (1995) The Nijmegen modification of the Bethesda assay for factor VIII:C inhibitors: improved specificity and reliability.Thromb Haemost 73:247–51.
 
 
 
 
 
Jundishapur Scientific Medical Journal
Volume 16, Issue 3 - Serial Number 108
September and October 2017
Pages 326-332

Files

History

  • Receive Date: 13 March 2017
  • Revise Date: 14 June 2017
  • Accept Date: 23 July 2017

Share

How to cite

Statistics