Reclassifying hemophilia to include the definition of outcomes and phenotype as new targets

Jecko Thachil; Jean M Connors; Johnny Mahlangu; Michelle Sholzberg

doi:10.1016/j.jtha.2023.03.016

Reclassifying hemophilia to include the definition of outcomes and phenotype as new targets

J Thromb Haemost. 2023 Jul;21(7):1737-1740. doi: 10.1016/j.jtha.2023.03.016. Epub 2023 Mar 25.

Authors

Jecko Thachil¹, Jean M Connors², Johnny Mahlangu³, Michelle Sholzberg⁴

Affiliations

¹ Department of Haematology, Manchester University Hospitals, Oxford Road, Manchester, UK. Electronic address: jecko.thachil@mft.nhs.uk.
² Hematology Division, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA.
³ Department of Molecular Medicine and Haematology, School of Pathology, University of the Witwatersrand and the National Health Laboratory Service, Johannesburg, South Africa.
⁴ Departments of Medicine, and Laboratory Medicine and Pathobiology, St. Michael's Hospital, Li Ka Shing Knowledge Institute, University of Toronto, Toronto, Ontario, Canada; St Michael's Hospital, Departments of Medicine and Laboratory Medicine & Pathobiology.

PMID: 36972786
DOI: 10.1016/j.jtha.2023.03.016

Abstract

Hemophilia is classified into mild, moderate, and severe based on coagulation factor activity levels. Factor replacement and prophylaxis regimens in persons with hemophilia have helped to reduce bleeding and its related complications. With several newer treatments, some already approved and others soon to be, there may be a need to consider health-related quality of life in addition to bleed prevention as the goals of providing comprehensive care to persons with hemophilia. In this article, we discussed the reasons why such an approach may be relevant and call for the International Society of Thrombosis and Haemostasis to revisit the current classification of hemophilia.

Keywords: bleeding; classification; hemophilia; symptoms.

Publication types

Review

MeSH terms

Factor VIII / therapeutic use
Hemophilia A* / diagnosis
Hemophilia A* / drug therapy
Hemophilia B* / drug therapy
Hemorrhage / drug therapy
Humans
Phenotype
Quality of Life

Substances

Factor VIII