Volume 11, Issue 2 (Summer 2014)
bloodj 2014, 11(2): 110-115 |
Back to browse issues page
Download citation:
BibTeX | RIS | EndNote | Medlars | ProCite | Reference Manager | RefWorks
Send citation to:
BibTeX | RIS | EndNote | Medlars | ProCite | Reference Manager | RefWorks
Send citation to:
Fatehifar M, 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. bloodj 2014; 11 (2) :110-115
URL: http://bloodjournal.ir/article-1-877-en.html
URL: http://bloodjournal.ir/article-1-877-en.html
Full-Text [PDF 284 kb]
(4414 Downloads)
| Abstract (HTML) (16351 Views)
The correlation of factor VIII and von Willebrand levels
with clinical symptoms in obligate carriers of
hemophilia A in Isfahan
Fatehifar M.R.1,2, Horfar H.3, Akbari N.1,2, Eshraghi M.4, Hojatifar M.5,
Motamedi N.5, Shahinpour Z.1,2
1Blood Transfusion Research Center, High Institute for Research and Education in Transfusion Medicine, Tehran, Iran
2Isfahan Regional Educational Blood Transfusion Center, Isfahan, Iran
3Isfahan University of Medical Sciences, Isfahan, Iran
4Comprehensive Haemophilia Treatment Center, Tehran, Iran
5Seyed-al-Shohada Hospital, Isfahan University of Medical Sciences, Isfahan, Iran
Abstract
Background and Objectives
Bleeding tendency in obligate carriers of hemophilia A has been found since several years ago; some of haemophiliacs with factor levels of 40-60% also have abnormal bleeding. The present study is to measure factor VIII and von Willebrand levels and evaluate their correlation with clinical finding in obligate carriers.
Materials and Methods
In this cross-sectional study conducted 49 obligate carriers of Haemophilia A. F.VIII, vWF: Ag, PT, and APTT levels were checked and their correlationship with the bleeding score was evaluated and analyzed.
Results
Fourty-Nine Obligate carriers included in the study. The mean of PT, PTT , F.VIII, vWF: Ag and bleeding score (BS) were 12.6 ± 1.3, 36.3 ± 4.2, 52.5 ± 22.6, 83.7 ± 35.2, and 1.5 ± 1.7, respectively. The present study showed only 4% (2) of the carriers having had bleeding score (BS) equal to 5 and higher. Only F.VIII / vWF: Ag positively correlated with BS significantly (p = 0.006).
Conclusions
The present study showed that PT, PTT, and F.VIII levels and the bleeding score alone could not be helpful in screening obligate carriers. It seems that only determination of F.VIII/vWF: Ag ratio be helpful hn carrier states. But definitive diagnosis carrier states is by molecular genetic testing.
Key words: IRAN, Hemophilia A, Hemorrhage, Carrier State
Received: 15 Apr 2012
Accepted: 11 Jan 2014
Correspondence: Akbari N., MD. Blood Transfusion Research Center, High Institute for Research and Education in
Transfusion Medicine and Isfahan Regional Educational Blood Transfusion Center.
P.O. Box: 81688-44861, Isfahan, Iran. Tel: (+98311) 2607074; Fax: (+98311) 2607074
E-mail: nah_akbari@yahoo.com
Full-Text: (7073 Views)
References:
- Merskey C. The occurrence of haemophilia in the human female. Q J Med 1951; 20(79): 299-312.
- Ross J. Perspectives of haemophilia carriers. Haemophilia 2000; 6 Suppl 1: 41-5.
- Mauser-Bunschoten EP. Symptomatic carriers of Hemophilia. World Federation of Hemophilia 2008; 46: 2-3
- Kadir RA, Economides DL, Braithwaite J, Goldman E, Lee CA. The obstetric experience of carriers of haemophilia. Br J Obstet Gynaecol 1997; 104(7): 803-10.
- Fishman DJ, Jones PK, Menitove JE, Ratnoff OD, Everson B. Detection of the carrier state for classic hemophilia using an enzyme-linked immunosorbent assay (ELISA). Blood 1982; 59(6): 1163-8.
- 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.
- Rodeghiero F, Tosetto A, Castaman G. How to estimate bleeding risk in mild bleeding disorders. J Thromb Haemost 2007; 5 Suppl 1: 157-66.
- Street AM, Ljung RL, Lavery SA. Management of carriers and babies with haemophilia. Haemophilia 2008; 14 Suppl 3: 181-7.
- Tosetto A, Castaman G, Rodeghiero F. Bleeding scores in inherited bleeding disorders: Clinical or research tools? Haemophilia 2008; 14(3): 415-22.
- 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.
- 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.
- Winikoff R, Lee C. Hemophilia carrier status and counseling the symptomatic and asymptomatic adolescent. J Pediatr Adolesc Gynecol 2010; 23(6 Suppl): S43-7.
- 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.
- Ratnoff OD, Jones PK. The laboratory diagnosis of the carrier state for classic hemophilia. Ann Intern Med 1977; 86(5): 521-8.
- 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.
- 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.
|
Original Article
|
|
Sci J Iran Blood Transfus Organ 2014; 11(2): 110-115 |
The correlation of factor VIII and von Willebrand levels
with clinical symptoms in obligate carriers of
hemophilia A in Isfahan
Fatehifar M.R.1,2, Horfar H.3, Akbari N.1,2, Eshraghi M.4, Hojatifar M.5,
Motamedi N.5, Shahinpour Z.1,2
1Blood Transfusion Research Center, High Institute for Research and Education in Transfusion Medicine, Tehran, Iran
2Isfahan Regional Educational Blood Transfusion Center, Isfahan, Iran
3Isfahan University of Medical Sciences, Isfahan, Iran
4Comprehensive Haemophilia Treatment Center, Tehran, Iran
5Seyed-al-Shohada Hospital, Isfahan University of Medical Sciences, Isfahan, Iran
Abstract
Background and Objectives
Bleeding tendency in obligate carriers of hemophilia A has been found since several years ago; some of haemophiliacs with factor levels of 40-60% also have abnormal bleeding. The present study is to measure factor VIII and von Willebrand levels and evaluate their correlation with clinical finding in obligate carriers.
Materials and Methods
In this cross-sectional study conducted 49 obligate carriers of Haemophilia A. F.VIII, vWF: Ag, PT, and APTT levels were checked and their correlationship with the bleeding score was evaluated and analyzed.
Results
Fourty-Nine Obligate carriers included in the study. The mean of PT, PTT , F.VIII, vWF: Ag and bleeding score (BS) were 12.6 ± 1.3, 36.3 ± 4.2, 52.5 ± 22.6, 83.7 ± 35.2, and 1.5 ± 1.7, respectively. The present study showed only 4% (2) of the carriers having had bleeding score (BS) equal to 5 and higher. Only F.VIII / vWF: Ag positively correlated with BS significantly (p = 0.006).
Conclusions
The present study showed that PT, PTT, and F.VIII levels and the bleeding score alone could not be helpful in screening obligate carriers. It seems that only determination of F.VIII/vWF: Ag ratio be helpful hn carrier states. But definitive diagnosis carrier states is by molecular genetic testing.
Key words: IRAN, Hemophilia A, Hemorrhage, Carrier State
Received: 15 Apr 2012
Accepted: 11 Jan 2014
Correspondence: Akbari N., MD. Blood Transfusion Research Center, High Institute for Research and Education in
Transfusion Medicine and Isfahan Regional Educational Blood Transfusion Center.
P.O. Box: 81688-44861, Isfahan, Iran. Tel: (+98311) 2607074; Fax: (+98311) 2607074
E-mail: nah_akbari@yahoo.com
Type of Study: Research |
Subject:
Hematology
Send email to the article author
| Rights and permissions | |
 |
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License. |
