Abstract
Aktive protrombin kompleks konsantresi (aPCC) ve rekombinant faktör VIIa (rFVIIa) inhibitör geliştirmiş hemofili hastalarının kanama tedavisinde ve profilaksisinde kullanılmaktadır. Ülkemizde rFVIIa için profilakside endikasyon onayı ve geri ödemesi henüz yoktur. Bu yazıda inhibitörlü hastada aPCC’nin doz ve etkinliği değerlendirilmiştir. Her üç vakamızda da aPCC profilaksisi alırken kanamanın olmaması, iki hastada profilaksi kesildikten sonra tekrarlayan kanamaların görülmesi profilaksi süresinin uzatılabileceğini desteklemektedir.
Keywords
References
- Kavaklı K, Şahin F, Demir M, THD Hemofili Çalışma Grubu. He- mofilide İnhibitör Sorunu Tanı ve Tedavi Kılavuzu, Ulusal Tedavi Kılavuzu. 2011; IV. Bölüm: 45-54.
- Mathev P. Current opinion on inhibitor treatment options. Semin Hematol 2006;43(2 Suppl 4):S8-13.
- Lessinger C, Gringeri A, Antmen B, Berntrop E, Biasoli C, Car- penter S, et al. Anti- inhibitor Coagulant Complex Prophylaxis in Hemophilia with İnhibitors. N Engl J Med 2011;365(18):1684-92.
- de Paula EV, Kavakli K, Mahlangu J, Ayob Y, Lentz SR, Morfi- ni M, et al. Recombinate factor VIIa analog (vaterptacog alfa [ac- tivated] for treatment of joint bleeds in hemophilia patients with inhibitors: a randomized controlled trial. J Thromb Haemost. 2012;10(1):81-9.
- Maclean PS, Richards M, Williams M, Collins P, Liesner R, et al. Treatment related factors and inhibitors development in children with severe hemophilia A. Haemophilia 2011;17(2):282-7.
- Dimichele D, Negrier C. A retrospective postlicensure survey of FEIBA efficacy and safety. Haemophilia 2006;12(4):352-62.
- Valentino LA. Assing the benefits of FEIBA prophylaxis in He- mophilia Patients with Inhibitors: A Meta-Analysis. Haemophilia 2008;14(Suppl 2):54(08 PO 73).
- Jimanez- Yuste V, Radriguez_ Merchan EC, Alvarez MT, Quintana M, Martin-Salces M, Hernandez-Navarro F. Eperiences in the pre- vention of arthrophaty in hemophilia patients with inhibitors. Hae- mophilia 2008;14 Suppl 6:28-35.
- Ewenstein B, Giangrande P, Morfini M,Tjonnfjord GE, Kraut E, Luu H. Evaluation of FEIBA for prophylaxis in patients with inhi- bitors. Haemophilia 2004; 10(Suppl 3): 114(22 PO 10).
- Ewing NP, Pullens L, De Guzman C. Anamnesis in patients with hemophilia and inhibitors who receive activated prothrombin complex concentrates for prophylaxis. J Thromb Hemost 2007; 5(Suppl 2): (P_T_158).
- Negrier C, Goudemand J, Sultan Y, Bertrand M, Rothschild C, La- uroua P. Multicenter retrospective study on the utilization of FE- IBA in France in patients with factor VIII and factor IX inhibi- tors. French FEIBA study Group, Factor Eight Bypassing Activity. Thromb Haemost 1997;77(6):1113-9.
Abstract
Use of activated prothrombin complex concentrate (aPCC) and recombinant factor VIIa (rFVIIa) is indicated for the treatment and prophylaxis of hemophiliac patients who has developed inhibitors. rFVIIa is an off labeled drug for prophylaxis in Turkey and not covered by health insurance. In this manuscript, the efficacy and dosage for aPCC prophylaxis were evaluated. During the aPCC prophylaxis, bleeding did not occur in any of the three cases. However, two patients developed recurrent bleeding attacks after the prophylaxis ceased. Therefore, we suggest that prophylaxis by aPCC should be prolonged to prevent recurrent bleeding attacks in hemophiliacs who developed inhibitors
Keywords
References
- Kavaklı K, Şahin F, Demir M, THD Hemofili Çalışma Grubu. He- mofilide İnhibitör Sorunu Tanı ve Tedavi Kılavuzu, Ulusal Tedavi Kılavuzu. 2011; IV. Bölüm: 45-54.
- Mathev P. Current opinion on inhibitor treatment options. Semin Hematol 2006;43(2 Suppl 4):S8-13.
- Lessinger C, Gringeri A, Antmen B, Berntrop E, Biasoli C, Car- penter S, et al. Anti- inhibitor Coagulant Complex Prophylaxis in Hemophilia with İnhibitors. N Engl J Med 2011;365(18):1684-92.
- de Paula EV, Kavakli K, Mahlangu J, Ayob Y, Lentz SR, Morfi- ni M, et al. Recombinate factor VIIa analog (vaterptacog alfa [ac- tivated] for treatment of joint bleeds in hemophilia patients with inhibitors: a randomized controlled trial. J Thromb Haemost. 2012;10(1):81-9.
- Maclean PS, Richards M, Williams M, Collins P, Liesner R, et al. Treatment related factors and inhibitors development in children with severe hemophilia A. Haemophilia 2011;17(2):282-7.
- Dimichele D, Negrier C. A retrospective postlicensure survey of FEIBA efficacy and safety. Haemophilia 2006;12(4):352-62.
- Valentino LA. Assing the benefits of FEIBA prophylaxis in He- mophilia Patients with Inhibitors: A Meta-Analysis. Haemophilia 2008;14(Suppl 2):54(08 PO 73).
- Jimanez- Yuste V, Radriguez_ Merchan EC, Alvarez MT, Quintana M, Martin-Salces M, Hernandez-Navarro F. Eperiences in the pre- vention of arthrophaty in hemophilia patients with inhibitors. Hae- mophilia 2008;14 Suppl 6:28-35.
- Ewenstein B, Giangrande P, Morfini M,Tjonnfjord GE, Kraut E, Luu H. Evaluation of FEIBA for prophylaxis in patients with inhi- bitors. Haemophilia 2004; 10(Suppl 3): 114(22 PO 10).
- Ewing NP, Pullens L, De Guzman C. Anamnesis in patients with hemophilia and inhibitors who receive activated prothrombin complex concentrates for prophylaxis. J Thromb Hemost 2007; 5(Suppl 2): (P_T_158).
- Negrier C, Goudemand J, Sultan Y, Bertrand M, Rothschild C, La- uroua P. Multicenter retrospective study on the utilization of FE- IBA in France in patients with factor VIII and factor IX inhibi- tors. French FEIBA study Group, Factor Eight Bypassing Activity. Thromb Haemost 1997;77(6):1113-9.
Details
| Other ID | JA99SE63BE |
|---|---|
| Journal Section | Case Report |
| Authors | |
| Publication Date | April 1, 2012 |
| Submission Date | April 1, 2012 |
| Published in Issue | Year 2012 Volume: 6 Issue: 4 |
Cite
The publication language of Turkish Journal of Pediatric Disease is English.
Manuscripts submitted to the Turkish Journal of Pediatric Disease will go through a double-blind peer-review process. Each submission will be reviewed by at least two external, independent peer reviewers who are experts in the field, in order to ensure an unbiased evaluation process. The editorial board will invite an external and independent editor to manage the evaluation processes of manuscripts submitted by editors or by the editorial board members of the journal. The Editor in Chief is the final authority in the decision-making process for all submissions. Articles accepted for publication in the Turkish Journal of Pediatrics are put in the order of publication taking into account the acceptance dates. If the articles sent to the reviewers for evaluation are assessed as a senior for publication by the reviewers, the section editor and the editor considering all aspects (originality, high scientific quality and citation potential), it receives publication priority in addition to the articles assigned for the next issue.
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