Literature DB >> 31529382

Hemophilia - Impact of Recent Advances on Management.

Geetha Puthenveetil1,2, Diane Nugent3,4,5.   

Abstract

There have been numerous advances in the field of hemophilia management in the past decade, including long acting factor products, non-factor products, and potentially curative interventions such as gene therapy. Each of these interventions introduces exciting treatment modalities to patients with both hemophilia A and B, however they also pose a daunting array of possible management options. Adverse reactions to novel agents are being reported as more patients are treated and long-term sustainability of interventions such as gene therapy is yet to be determined. The practicing hematologist should be aware of the intricacies involved in customizing care for their individual patients and be aware of the monitoring strategies for each interventional strategy to avoid adverse events. Upfront cost vs. long term benefit should be considered as choices of treatment strategies are made, especially in resource poor countries. The goal of the newer agents is to decrease annualized bleed rates and avoid debilitating arthropathy. This article looks at current treatment models for prophylaxis and management of inhibitors, reviews the recent advances in the field (with bioengineered factor products, non-factor products and gene therapy) and summarizes the incorporation of these new interventions in the treatment plan for patients with hemophilia.

Entities:  

Keywords:  Extended half-life products; Gene therapy; Hemophilia; Non-factor replacement products

Mesh:

Substances:

Year:  2019        PMID: 31529382     DOI: 10.1007/s12098-019-03062-8

Source DB:  PubMed          Journal:  Indian J Pediatr        ISSN: 0019-5456            Impact factor:   1.967


  35 in total

1.  Twenty-five years' experience of prophylactic treatment in severe haemophilia A and B.

Authors:  I M Nilsson; E Berntorp; T Löfqvist; H Pettersson
Journal:  J Intern Med       Date:  1992-07       Impact factor: 8.989

Review 2.  Life in the shadow of a dominant partner: the FVIII-VWF association and its clinical implications for hemophilia A.

Authors:  Steven W Pipe; Robert R Montgomery; Kathleen P Pratt; Peter J Lenting; David Lillicrap
Journal:  Blood       Date:  2016-09-01       Impact factor: 22.113

3.  Complete correction of hemophilia B phenotype by FIX-Padua skeletal muscle gene therapy in an inhibitor-prone dog model.

Authors:  Robert A French; Benjamin J Samelson-Jones; Glenn P Niemeyer; Clinton D Lothrop; Elizabeth P Merricks; Timothy C Nichols; Valder R Arruda
Journal:  Blood Adv       Date:  2018-03-13

4.  Recombinant factor VIII Fc fusion protein for immune tolerance induction in patients with severe haemophilia A with inhibitors-A retrospective analysis.

Authors:  M Carcao; A Shapiro; J M Staber; N Hwang; C Druzgal; K Lieuw; M Belletrutti; C D Thornburg; S P Ahuja; J Morales-Arias; J Dumont; G Miyasato; E Tsao; N Jain; S W Pipe
Journal:  Haemophilia       Date:  2018-02-13       Impact factor: 4.287

5.  Targeting of Antithrombin in Hemophilia A or B with RNAi Therapy.

Authors:  K John Pasi; Savita Rangarajan; Pencho Georgiev; Tim Mant; Michael D Creagh; Toshko Lissitchkov; David Bevan; Steve Austin; Charles R Hay; Inga Hegemann; Rashid Kazmi; Pratima Chowdary; Liana Gercheva-Kyuchukova; Vasily Mamonov; Margarita Timofeeva; Chang-Heok Soh; Pushkal Garg; Akshay Vaishnaw; Akin Akinc; Benny Sørensen; Margaret V Ragni
Journal:  N Engl J Med       Date:  2017-07-10       Impact factor: 91.245

6.  Immunogenicity, efficacy and safety of Nuwiq® (human-cl rhFVIII) in previously untreated patients with severe haemophilia A-Interim results from the NuProtect Study.

Authors:  R J Liesner; M Abashidze; O Aleinikova; C Altisent; M J Belletrutti; A Borel-Derlon; M Carcao; H Chambost; A K C Chan; L Dubey; J Ducore; N A Fouzia; M Gattens; Y Gruel; B Guillet; N Kavardakova; M El Khorassani; A Klukowska; T Lambert; S Lohade; M Sigaud; V Turea; J K M Wu; V Vdovin; A Pavlova; M Jansen; L Belyanskaya; O Walter; S Knaub; E J Neufeld
Journal:  Haemophilia       Date:  2017-08-16       Impact factor: 4.287

7.  A randomized study of very low-dose factor VIII prophylaxis in severe haemophilia - A success story from a resource limited country.

Authors:  S P Verma; T K Dutta; S Mahadevan; P Nalini; D Basu; N Biswal; A Ramesh; D Charles; K V Vinod; K T Harichandra Kumar
Journal:  Haemophilia       Date:  2016-03-14       Impact factor: 4.287

8.  A randomized clinical trial of prophylaxis in children with hemophilia A (the ESPRIT Study).

Authors:  A Gringeri; B Lundin; S von Mackensen; L Mantovani; P M Mannucci
Journal:  J Thromb Haemost       Date:  2011-04       Impact factor: 5.824

9.  Selection and evaluation of clinically relevant AAV variants in a xenograft liver model.

Authors:  Leszek Lisowski; Allison P Dane; Kirk Chu; Yue Zhang; Sharon C Cunningham; Elizabeth M Wilson; Sean Nygaard; Markus Grompe; Ian E Alexander; Mark A Kay
Journal:  Nature       Date:  2013-12-25       Impact factor: 49.962

Review 10.  Adeno-Associated Virus (AAV) as a Vector for Gene Therapy.

Authors:  Michael F Naso; Brian Tomkowicz; William L Perry; William R Strohl
Journal:  BioDrugs       Date:  2017-08       Impact factor: 5.807

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