Guidelines for the Management of Hemophilia


Year: 2020

Language: English

Author(s): Alok Srivastava et al on behalf of the WFH Guidelines for the Management of Hemophilia panelists and co-authors

Also Available In: Spanish, Russian, French, Simplified Chinese, Arabic

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Additional Information

The second edition of the Guidelines has also been translated into the following languages, with permission from the WFH, by NMOs: AzerbaijaniPolish, Traditional Chinese

The third edition of the WFH Guidelines for the Management of Hemophilia has just been published in the journal, Haemophilia! Click here to access the journal publication.

The contents of this page and the eLearning Platform will be updated to the 3rd edition of the guidelines over the coming weeks. The English version will be updated first and the French and Spanish will follow.

This new edition of the World Federation of Hemophilia (WFH) guidelines for the management of hemophilia comes at an exciting time in the evolution of the diagnosis and treatment of this condition. Since the publication of the second edition in 2012, tremendous advances have been made in several aspects of the management of hemophilia. These include genetic assessment as well as therapy with many innovative therapeutic products including extended half-life factor VIII (FVIII) and factor IX (FIX) products, a bispecific antibody, and hemostasis rebalancing drugs now in clinical development. All of these allow for more effective hemostasis than was possible in the past. Laboratory monitoring of therapies is better defined and prophylaxis is accepted as the only way to change the natural history of bleeding. There are highly effective therapies for patients with inhibitors. Outcome assessment with validated clinimetric instruments is widely advocated and practiced. All these advances are reflected in this third edition of the WFH guidelines, with new chapters devoted to several of these topics along with a new chapter on principles of care that aims to provide a framework for development of a comprehensive healthcare system for hemophilia including advocacy and empowerment for people with hemophilia (PWH). The recommendations in this edition were all developed through a formal evidence-informed and consensus-based methodology involving multidisciplinary healthcare professionals (HCPs) and well-informed PWH. While directed primarily at HCPs, these guidelines should also be very useful for PWH as well as advocacy organizations.

This third edition of the WFH Guidelines for the Management of Hemophilia has been endorsed by the Asian-Pacific Society on Thrombosis and Hemostasis, European Haemophilia Consortium, and National Hemophilia Foundation (USA).

Read the editorial on the guidelines, by Cedric Hermans and Mike Makris, in Haemophilia entitled: ‘Haemophilia Guidelines for All’: A new ambition for the World Federation of Hemophilia (WFH).

The WFH recently endorsed the NHF-McMaster Guideline on Care Models for Hemophilia Management. Click here to view the full text of the guideline, published in the Journal of Haemophilia, June 27, 2016, or here to read the Executive Summary of the NHF-McMaster guideline.

Content, 3rd edition

Explore the individual chapters of the 3rd edition of the WFH Guidelines for the Management of Hemophilia.

  Summary and introduction

  Chapter 1: Principles of Care

1.1 Principle 1: National coordination and delivery of hemophilia care 1.7 Principle 7: Multidisciplinary care for hemophilia Table 1‑1 Roles of hemophilia comprehensive care centres and hemophilia treatment centres
1.2 Principle 2: Access to safe CFCs, other hemostasis products, and curative therapies 1.8 Principle 8: Regular replacement therapy (prophylaxis)
1.3 Principle 3: Laboratory services and genetic diagnosis of hemophilia 1.9 Principle 9: Management of patients with inhibitors
1.4 Principle 4: Education and training in hemophilia care 1.10 Principle 10: Management of musculoskeletal complications
1.5 Principle 5: Clinical and epidemiological research 1.11 Principle 11: Management of specific conditions and comorbidities
1.6 Principle 6: Acute and emergency care for bleeds 1.12 Principle 12: Outcome assessment


2.1 Introduction 2.5 Home therapy Table 2‑1 Relationship of bleeding severity to clotting factor level
2.2 Comprehensive care 2.6 Pain management Table 2‑2 Sites of bleeding in hemophilia
2.3 Fitness and physical activity 2.7 Dental care and management Table 2‑3 Approximate frequency of bleeding at different sites
2.4 Adjunctive management 2.8 Transition from pediatric to adult care Table 2‑4 Pain management strategies for people with hemophilia


3.1 Introduction 3.3 Use of the correct equipment and reagents Table 3‑1 Interpretation of screening tests
3.2 Coagulation laboratory testing 3.4 Quality assurance Table 3‑2 Publications with data related to the use of different FVIII assays in the presence of recombinant and modified factor VIII concentrates
Table 3‑3 Publications with data related to the use of different FIX assays in the presence of recombinant and modified factor IX concentrates


  Chapter 4: Genetic Assessment

4.1 Introduction
4.2 Indications for genetic assessment
4.3 Strategy for genetic testing of probands
4.4 Techniques for genetic assessment
4.5 Classification and description of variants
4.6 Interpretive reports
4.7 Strategies if causative variant is not detected
4.8 Quality assurance

  Chapter 5: Hemostatic Agents

5.1 Introduction
5.2 Product selection
5.3 Clotting factor concentrates (CFCs)
5.4 Bypassing agents
5.5 Other plasma products
5.6 Other pharmacological options
5.7 Non-factor replacement therapies


6.1 Introduction 6.7 Tailored factor prophylaxis regimens Table 6‑1 Conventional factor prophylaxis for hemophilia A and B defined according to when prophylaxis is initiated
6.2 Benefits of prophylaxis 6.8 Adherence and patient/caregiver education Table 6‑2 Conventional factor prophylaxis with standard half-life clotting factor defined according to its intensity
6.3 Standard half-life factor prophylaxis 6.9 Health economics of prophylaxis Table 6‑3 Variables that affect factor levels (applies to both SHL and EHL clotting factors) in people with hemophilia
6.4 Extended half-life factor prophylaxis 6.10 Low-dose prophylaxis for patients with limited access of CFCs Table 6‑4 Documented benefits of EHL CFCs
6.5 Prophylaxis with non-factor replacement therapy 6.11 New definitions of prophylaxis Table 6‑5 Advantages and disadvantages of fixed “one size fits all” SHL factor prophylaxis regimens
6.6 Fixed/non-tailored factor prophylaxis regimens 6.12 Future research questions to be addressed Table 6‑6 Tailoring prophylaxis to patient needs
Table 6-7 Factors that affect bleeding phenotype and contribute to inter-patient phenotypic variability


7.1 Introduction 7.7 Ophthalmic hemorrhage Table 7‑1 Definitions of response to treatment
7.2 Joint hemorrhage 7.8 Oral hemorrhage Table 7‑2 Practice patterns: peak plasma factor levels and duration of administration
7.3 Central nervous system and intracranial hemorrhage 7.9 Epistaxis
7.4 Throat and neck hemorrhage 7.10 Lacerations and abrasions
7.5 Gastrointestinal/abdominal hemorrhage 7.11 Soft tissue hemorrhage
7.6 Renal hemorrhage 7.12 Practice patterns in CFC replacement


8.1 Introduction 8.3 Hemophilia A and FVIII inhibitors Table 8‑1 Indications for inhibitor testing
8.2 Inhibitor screening 8.4 Hemophilia B and FIX inhibitors Table 8‑2 Potential risk factors for inhibitors
Table 8-3 Treatment of acute bleeds in hemophilia A patients with inhibitors
Table 8-4 Sequential bypass agent therapy alternating rFVIIa and aPCC
Table 8-5 Treatment of acute bleeds in hemophilia B patients with inhibitors


9.1 Introduction 9.6 Sexuality Table 9‑1 Definition of adequacy of hemostasis for surgical procedures
9.2 Carriers 9.7 Psychosocial issues
9.3 Circumcision 9.8 Comorbidities
9.4 Vaccinations 9.9 Medical issues with aging
9.5 Surgery and invasive procedures


10.1 Introduction 10.6 Fractures
10.2 Synovitis 10.7 Orthopedic surgery in hemophilia
10.3 Hemophilic arthropathy 10.8 Joint replacement
10.4 Muscle hemorrhage 10.9 Psychosocial impacts of musculoskeletal complications
10.5 Pseudotumours


11.1 Introduction 11.6 Economic factors Figure 11‑1 International Classification of Functioning and Health (ICF) model
11.2 Outcome assessment in hemophilia 11.7 Health-related quality of life Figure 11‑2 Hemophilia Joint Health Score 2.1 – Summary Score Sheet
11.3 Body structure and function 11.8 Patient-reported outcomes Table 11‑1 Radiological Pettersson score
11.4 Activities and participation 11.9 Core set of measures for use in the clinic or research setting Table 11‑2 IPSG MRI Scale to Assess Hemophilic Arthropathy
11.5 Environmental and personal factors Table 11-3 HEAD-US Scoring Method
Table 11-4 Haemophilia Activities List (HAL) 200515
Table 11-5 Haemophilia Activities List —Pediatric (PedHAL) v.11
Table 11-6 Functional Independence Score in Hemophilia (FISH)
Table 11-7 Q-5D Instrument
Table 11-8 36-Item Short Form Survey Instrument (SF-36)


12.1 Background 12.5 Finalization of the recommendations and manuscript development
12.2 Methodology 12.6 Methodology limitations
12.3 Evidence generation 12.7 Future plans for updates
12.4 Formal consensus achievement through Delphi techniques 12.8 Conclusion


eLearning Modules, 2nd edition

Explore the 6 sections of the 2nd edition of the WFH Guidelines for the Management of Hemophilia in 6 dynamic illustrated eLearning modules in English, Spanish, and French!

Each module consists of the text of one section of the WFH Guidelines for the Management of Hemophilia, brought to life with detailed colour illustrations, interactive features, a glossary of terms, and linked resources for further reading. There are even quizzes, and progress checks so that you can evaluate whether you have fully understood the content. You can work through an entire module or look up sections of particular interest in the table of contents. These eLearning modules render the complex and important information in the WFH Guidelines for the Management of Hemophilia accessible to everyone.