Management of Musculoskeletal Issues in Hemophiliacs at a Glance

Introduction

Hemophilia is characterized by acute bleeding, which in up to 80% of cases involves joint bleeding. In severe forms of hemophilia, spontaneous bleeding or bleeding in relation to minimal trauma occurs. Repeated bleeding leads to progressive joint damage, responding to the presence of blood in the joint, which triggers an inflammatory response.

Hemarthrosis, Synovitis, Hemophilic Arthropathy

Acute hemarthrosis is followed by synovial hyperemia, making it inflamed and fragile. Inadequate treatment of acute synovitis and bleeding results in continuous synovial reaction, repeated bleeding, and damage to joint structures. A process lasting more than 3 months is referred to as chronic synovitis. The team caring for hemophiliacs must actively search for signs of synovitis, using not only clinical joint assessment but also tools like ultrasound.

In cases of persistent chronic synovitis, the WFH guidelines recommend non-surgical synovectomy using radioisotopes as the first line of treatment. After radiosynoviorthesis (RSO), the joint should be immobilized for at least 24 hours, followed by progressive rehabilitation aimed at restoring its strength and function. Other options include chemical synoviorthesis or selective embolization of vessels supplying the synovium. In case of surgical synovectomy, the arthroscopic method is preferred.

Chronic arthropathy has severe consequences on daily life—limiting mobility, leading to chronic debilitating pain, disrupting social interactions, and restricting workforce participation.

The primary component in the prevention and treatment of chronic hemophilic arthropathy is prophylactic therapy utilizing factor concentrates or non-factor treatments. Physiotherapy, often supported by prophylaxis, is also essential.

Muscle Bleeding

In the event of muscle bleeding, aside from factor concentrate therapy, physiotherapy is crucial. It should begin as soon as the pain subsides and should gradually work towards restoring muscle length, strength, and function to prevent post-hemorrhagic contractures. A particularly significant muscle bleed occurs in the musculus iliopsoas area, not only due to its diagnostic challenge but also because inadequate treatment can have serious consequences regarding pain and muscle function.

The Importance of Active Bleeding Prevention and Multidisciplinary Teams

Since it is known that people with hemophilia and chronic musculoskeletal issues perceive both functional and social limitations, it is essential to focus on the prevention and treatment of these issues and to ensure a multidisciplinary team that includes psychologists or pain management specialists, among others. These steps can effectively help mitigate the functional impact of potential bleeding episodes in practice.

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Source: Srivastava A., Santagostino E., Dougall A. et al. WFH Guidelines for the Management of Hemophilia, 3^rd edition. Haemophilia 2020; 26 (Suppl. 6): 1−158, doi: 10.1111/hae.14046.