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Standard Prophylactic Regimen Is Three Times and Twice a Week in Hemophilia A and B, Respectively. The Low Dose Tailoring Method of Prophylaxis Has Been Introduced for Patients with Hemophilia

1Hematology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran, Islamic Republic of, 2Shiraz Hemophilia Comprehensive Center, Dastgheib Hospital, Shiraz University of Medical Sciences, Shiraz, Iran, Islamic Republic of

Abstract Number: PB0631

Meeting: ISTH 2021 Congress

Theme: Hemophilia and Rare Bleeding Disorders » Hemophilia - Clinical

Background: Standard prophylactic regimen is three times and twice a week in hemophilia A and B, respectively. The low dose tailoring method of prophylaxis has been introduced for patients with hemophilia.

Aims: To evaluate the efficacy and safety of low dose modified prophylaxis, once per week in patients with severe hemophilia.

Methods: In this retrospective study, 53 patients less than 15 years with severe hemophilia (45 hemophilia A and 8 hemophilia B) who were involved in standard prophylactic regimen during 2015-2018 were investigated. All patients underwent prophylaxis with dose of 25 IU/kg once per week and increased to twice or 3 times a week if any of these conditions occurred:

  1. Three episodes of hemarthrosis in past three months 
  2. Four episodes of important soft tissue bleeding in past three months 
  3. Any episode of a life-threatening and major bleeding without history of trauma 

Results: All studied patients were negative for factor inhibitor. Mean age of diagnosis and mean age of starting infusion were 1.60 ± 1.41 (range: 1-7) and 2.71 ± 2.21 (range: 1-7) respectively. Mean age of starting prophylaxis was 6.15 ± 3.9 (range: 1-14) years. The duration of prophylaxis was 47.09 ± 11.12 months. Nine patients discontinued prophylaxis and were excluded. Mean annual bleeding rate in the remained patients (n=44) significantly decreased from 18.5 ± 10.3 episode before prophylaxis to 5.7 ± 3.7 episode after prophylaxis (P<0.001).
Number of patients received primary, secondary, and tertiary prophylaxis were 17 (32.1%), 33 (62.3%), and 3 (5.7%) respectively

Conclusions: Prophylactic treatment in patients with severe hemophilia is shown to significantly reduce the numbers of bleeding episodes, arthropathy and costs of hospitalization. Our results showed modified prophylaxis treatment as a first choice in patients with severe hemophilia, especially in developing countries facing with limitation of financial and medical resources would be an ideal optional treatment.

To cite this abstract in AMA style:

Karimi M, Parsa A, Haghpanah S, Sadat Rajaie Ramsheh F, Bordbar M, Bolandparvaz N, Bazrafshan A, Tavoosi H. Standard Prophylactic Regimen Is Three Times and Twice a Week in Hemophilia A and B, Respectively. The Low Dose Tailoring Method of Prophylaxis Has Been Introduced for Patients with Hemophilia [abstract]. Res Pract Thromb Haemost. 2021; 5 (Suppl 2). https://abstracts.isth.org/abstract/standard-prophylactic-regimen-is-three-times-and-twice-a-week-in-hemophilia-a-and-b-respectively-the-low-dose-tailoring-method-of-prophylaxis-has-been-introduced-for-patients-with-hemophilia/. Accessed December 11, 2023.

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