9  Treatment of bleeds


Eva Funding

Hemophilia Centre Copenhagen, Department of Hematology, Rigshospitalet, Copenhagen, Denmark

Nadine Gretenkort Andersson

Department for Paediatric Haematology and Oncology, Children’s Hospital, Skåne University Hospital, Malmö, Sweden

Marianne Hoffmann

Haemophilia Centre Copenhagen, Department of pediatrics, Rigshospitalet, Copenhagen, Denmark

Heidi Knudsen

Centre for Rare Disorders, Oslo University Hospital, Oslo, Norway

Minna Koskenvuo

Division of Hematology-Oncology and Stem Cell Transplantation, New Children’s Hospital, University of Helsinki and Helsinki University Hospital, Helsinki, Finland

Anna-Elina Lehtinen

Department of Hematology and Comprehensive Cancer Center, Unit of Coagulation Disorders, Helsinki University Hospital, Helsinki, Finland

Susanne Ranta

Childhood Cancer Research Unit, Department of Women’s and Children’s Health, Karolinska Institutet, Sweden and Astrid Lindgren Children’s Hospital, Karolinska University Hospital, Stockholm, Sweden

1 Introduction

  • Treatment only when acute bleeds occur is called treatment on demand. In mild and mild-moderate hemophilia spontaneous bleeds a rare, and patients are mostly treated on demand.

  • In severe and moderate-severe hemophilia, patients might suffer bleeds despite prophylactic treatment.

  • Acute bleeds are treated with factor products to target a factor level of 40-60 IU/dL for minor bleeds, and 70-100 IU/dL for major bleeds.

  • For potentially life-threatening bleeds such as head trauma, bleeding in the throat and neck and gastrointestinal bleeding the target is 70-100 IU/dL with repeated dosing every 6-8th hours until the bleeds resolves.

  • For further details on treatment of specific bleeds, see the WFH guideline https://guidelines.wfh.org/chapter/treatment-of-specific-hemorrhages/.

  • In mild hemophilia A, DDAVP should be tested as alternative to factor replacement therapy for treatment of acute bleeds as well as prophylaxis before surgery (see chapter Surgery in hemophilia).

  • For non-inhibitor patients in emicizumab prophylaxis, minor bleeds often resolve without treatment.

    • In case a minor bleed requires treatment, the recommended dose is 25 IU/kg.

    • Major bleeds should be treated with a dose of FVIII of 50 IU/kg and repeated dosing can be necessary.

    • The factor level can be measured with a chromogenic FVIII assay with bovine reagents.

  • For inhibitor patients, see chapter Inhibitors.


  • When treating minor bleeds, the target is a factor level of 40-60 IU/dL.


  • When treating major bleeds, the target is a factor level of 70-100 IU/dL.


  • DDAVP is recommended for on demand treatment in patients with mild hemophilia A.


  • Major or life-threatening bleeds under emicizumab prophylaxis in non-inhibitor patients should be treated with FVIII 50 IU/kg.