Literature DB >> 29328905

Diagnosis and care of patients with mild haemophilia: practical recommendations for clinical management.

Gary Benson1, Günter Auerswald2, Gerry Dolan3, Anne Duffy4, Cedric Hermans5, Rolf Ljung6,7, Massimo Morfini8, Silva Zupančić Šalek9,10,11.   

Abstract

Mild haemophilia is defined by factor levels between 0.05 and 0.40 IU/mL and is characterised by traumatic bleeds. Major issues associated with mild haemophilia are that it may not present for many years after birth, and that awareness, even within families, may be low. Methodological problems exist in diagnosis, such as inconsistencies in results obtained from different assays used to measure factor levels in mild haemophilia. Advances in genetic testing provide insight into diagnosis as well as the likelihood of inhibitor development, which is not uncommon in patients with mild or moderate haemophilia and can increase morbidity. The management of patients with mild haemophilia is a challenge. This review includes suggestions around formulating treatment plans for these patients, encompassing the full spectrum from clinical care of the newly diagnosed neonate to that of the ageing patient with multiple comorbidities. Management strategies consider not only the vast differences in these patients' needs, but also risks of inhibitor development and approaches to optimally engage patients.

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Year:  2017        PMID: 29328905      PMCID: PMC6214819          DOI: 10.2450/2017.0150-17

Source DB:  PubMed          Journal:  Blood Transfus        ISSN: 1723-2007            Impact factor:   3.443


  69 in total

1.  Haemophilia A carriers experience reduced health-related quality of life.

Authors:  L Gilbert; A Paroskie; D Gailani; M R Debaun; R F Sidonio
Journal:  Haemophilia       Date:  2015-04-30       Impact factor: 4.287

2.  Factor VIII inhibitors in mild and moderate-severity haemophilia A. UK Haemophilia Centre Directors Organisation.

Authors:  C R Hay; C A Ludlam; B T Colvin; F G Hill; F E Preston; N Wasseem; R Bagnall; I R Peake; E Berntorp; E P Mauser Bunschoten; K Fijnvandraat; C K Kasper; G White; E Santagostino
Journal:  Thromb Haemost       Date:  1998-04       Impact factor: 5.249

3.  Mild haemophilia: a disease with many faces and many unexpected pitfalls.

Authors:  K Peerlinck; M Jacquemin
Journal:  Haemophilia       Date:  2010-07       Impact factor: 4.287

4.  Clotting factor level is not a good predictor of bleeding in carriers of haemophilia A and B.

Authors:  Anna Olsson; Margareta Hellgren; Erik Berntorp; Rolf Ljung; Fariba Baghaei
Journal:  Blood Coagul Fibrinolysis       Date:  2014-07       Impact factor: 1.276

5.  The responsiveness of different APTT reagents to mild factor VIII, IX and XI deficiencies.

Authors:  A Bowyer; S Kitchen; M Makris
Journal:  Int J Lab Hematol       Date:  2010-09-14       Impact factor: 2.877

6.  DDAVP responsiveness in children with mild or moderate haemophilia A correlates with age, endogenous FVIII:C level and with haemophilic genotype.

Authors:  M E Seary; D Feldman; M D Carcao
Journal:  Haemophilia       Date:  2011-05-18       Impact factor: 4.287

Review 7.  Haemophilias A and B.

Authors:  Paula H B Bolton-Maggs; K John Pasi
Journal:  Lancet       Date:  2003-05-24       Impact factor: 79.321

8.  Diagnosis and management challenges in patients with mild haemophilia A and discrepant FVIII measurements.

Authors:  M Trossaert; A Lienhart; C Nougier; M Fretigny; M Sigaud; S Meunier; M Fouassier; C Ternisien; C Negrier; Y Dargaud
Journal:  Haemophilia       Date:  2014-02-12       Impact factor: 4.287

Review 9.  Inhibitor development in haemophilia B: an orphan disease in need of attention.

Authors:  Donna DiMichele
Journal:  Br J Haematol       Date:  2007-08       Impact factor: 6.998

10.  Prevalence and risk factors for hypertension in hemophilia.

Authors:  Annette von Drygalski; Nicholas A Kolaitis; Ricki Bettencourt; Jaclyn Bergstrom; R Kruse-Jarres; Doris V Quon; Christina Wassel; Ming C Li; Jill Waalen; Darlene J Elias; Laurent O Mosnier; Matthew Allison
Journal:  Hypertension       Date:  2013-04-29       Impact factor: 10.190

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  14 in total

Review 1.  Intracranial haemorrhage in children and adults with haemophilia A and B: a literature review of the last 20 years.

Authors:  Ezio Zanon; Samantha Pasca
Journal:  Blood Transfus       Date:  2018-02-04       Impact factor: 3.443

2.  Emicizumab for the treatment of haemophilia A: a narrative review.

Authors:  Massimo Franchini; Giuseppe Marano; Ilaria Pati; Fabio Candura; Samantha Profili; Eva Veropalumbo; Francesca Masiello; Liviana Catalano; Vanessa Piccinini; Stefania Vaglio; Simonetta Pupella; Giancarlo M Liumbruno
Journal:  Blood Transfus       Date:  2019-05       Impact factor: 3.443

3.  Challenges in the Diagnosis and Management of Non-Severe Hemophilia.

Authors:  Estera Boeriu; Teodora Smaranda Arghirescu; Margit Serban; Jenel Marian Patrascu; Eugen Boia; Cristian Jinca; Wolfgang Schramm; Adina Traila; Cristina Emilia Ursu
Journal:  J Clin Med       Date:  2022-06-09       Impact factor: 4.964

4.  Expert opinion on the UK standard of care for haemophilia patients with inhibitors: a modified Delphi consensus study.

Authors:  Kate Khair; Elizabeth Chalmers; Thuvia Flannery; Annabel Griffiths; Felicity Rowley; Guillermo Tobaruela; Pratima Chowdary
Journal:  Ther Adv Hematol       Date:  2021-04-30

Review 5.  Bridging the Missing Link with Emicizumab: A Bispecific Antibody for Treatment of Hemophilia A.

Authors:  Georg Gelbenegger; Christian Schoergenhofer; Paul Knoebl; Bernd Jilma
Journal:  Thromb Haemost       Date:  2020-07-27       Impact factor: 5.249

6.  Effect of Neurodynamics Nerve Flossing on Femoral Neuropathy in Haemophilic Patients: A randomized controlled study.

Authors:  Somaia A Hamed; Ibrahim M Zoheiry; Nevien Maher Waked; Lama Saad El-Din Mahmoud
Journal:  J Musculoskelet Neuronal Interact       Date:  2021-09-01       Impact factor: 2.041

7.  Surgical management of endometriosis in a severe Hemophilia A female patient and the role of transfusion medicine specialist: A case report with review of literature.

Authors:  Sanooja Pinki; Ganesh Mohan; Divya Venugopal; Susheela J Innah
Journal:  Asian J Transfus Sci       Date:  2021-11-01

Review 8.  IDELVION: A Comprehensive Review of Clinical Trial and Real-World Data.

Authors:  Miguel Escobar; Maria Elisa Mancuso; Cedric Hermans; Cindy Leissinger; Wilfried Seifert; Yanyan Li; William McKeand; Johannes Oldenburg
Journal:  J Clin Med       Date:  2022-02-18       Impact factor: 4.241

9.  Real-world data demonstrate improved bleed control and extended dosing intervals for patients with haemophilia B after switching to recombinant factor IX Fc fusion protein (rFIXFc) for up to 5 years.

Authors:  Amy Shapiro; Ateefa Chaudhury; Michael Wang; Miguel Escobar; Elisa Tsao; Christopher Barnowski; Jing Feng; Nisha Jain; Doris V Quon
Journal:  Haemophilia       Date:  2020-10-04       Impact factor: 4.287

10.  Haemophilia in France: Modelisation of the Clinical Pathway for Patients.

Authors:  Karen Beny; Benjamin du Sartz de Vigneulles; Florence Carrouel; Denis Bourgeois; Valérie Gay; Claude Negrier; Claude Dussart
Journal:  Int J Environ Res Public Health       Date:  2022-01-06       Impact factor: 3.390

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