Inherited coagulopathy
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| Section editors | |||
|---|---|---|---|
 |
Shruti Chaturvedi, MBBS, MSCI Baltimore, MD |
 |
Benjamin Tillman, MD Nashville, TN |
3 regimens on this page
3 variants on this page
|
Factor VIII deficiency (Hemophilia A), replacement products
To be completed
Factor VIII deficiency (Hemophilia A), with inhibitors, all lines of therapy
Emicizumab monotherapy
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Regimen
| Study | Evidence | Comparator | Efficacy |
|---|---|---|---|
| Oldenburg et al. 2017 (HAVEN 1) | Phase III (E) | No prophylaxis | Lower bleeding rate |
To be completed
Therapy
References
- HAVEN 1: Oldenburg J, Mahlangu JN, Kim B, Schmitt C, Callaghan MU, Young G, Santagostino E, Kruse-Jarres R, Negrier C, Kessler C, Valente N, Asikanius E, Levy GG, Windyga J, Shima M. Emicizumab prophylaxis in hemophilia A with inhibitors. N Engl J Med. 2017 Aug 31;377(9):809-818. Epub 2017 Jul 10. link to original article PubMed
Rituximab monotherapy
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Regimen
| Study | Evidence |
|---|---|
| Leissinger et al. 2014 | Phase II |
Immunosuppressive therapy
- Rituximab (Rituxan) 375 mg/m2 IV once per day on days 1, 8, 15, 22
28-day course
References
- Kempton CL, Allen G, Hord J, Kruse-Jarres R, Pruthi RK, Walsh C, Young G, Soucie JM. Eradication of factor VIII inhibitors in patients with mild and moderate hemophilia A. Am J Hematol. 2012 Sep;87(9):933-6. link to PMC article does not contain protocol PubMed
- Leissinger C, Josephson CD, Granger S, Konkle BA, Kruse-Jarres R, Ragni MV, Journeycake JM, Valentino L, Key NS, Gill JC, McCrae KR, Neufeld EJ, Manno C, Raffini L, Saxena K, Torres M, Marder V, Bennett CM, Assmann SF. Rituximab for treatment of inhibitors in haemophilia A: a phase II study. Thromb Haemost. 2014 Sep 2;112(3):445-58. link to original article PubMed
Factor IX deficiency (Hemophilia B), all lines of therapy
To be completed