Haemophilic arthropathy

Both factor VIII and factor IX haemophilia can cause, by repeated bleeding into the joints, a progressive joint destruction. Risk of bleed is predicted by the factor levels in the patients - spontaneous bleeds occur if the patient has levels of 5% or less of the normal levels.

The frequency and unpredictability of the bleeds in a haemophiliac pose a considerable disruption to life, and these ought to be considered when considering the impact of this disease on people.

This can be classified as; (1)

High risk - if severe haemophilia and not receiving prophylactic therapy (defined as the regular, continuous administration of a haemostatic agent/agents with the goal of preventing bleeding in people with haemophilia while allowing them to lead active lives and achieve quality of life comparable to non-haemophilia individuals).

Medium risk - if moderate haemophilia and not receiving prophylactic therapy.

Low risk - if mild haemophilia.

These complications may be prevented by the use of prophylactic factor VIII or factor IX infusions, or emicizumab for haemophilia A. (2)

Reference

1. Srivastava A, Santagostino E, Dougall A, et al. WFH guidelines for the management of hemophilia, 3rd edition. Haemophilia. 2020 Aug;26 Suppl 6:1-158.
2. Gualtierotti R, Solimeno LP, Peyvandi F. Hemophilic arthropathy: Current knowledge and future perspectives. J Thromb Haemost. 2021 Sep;19(9):2112-2121