Therapeutic options for hemarthrosis in severe hemophilic patients

Abstract

the coagulation factors VIII (FVIII) or IX (FIX), respectively. The primary therapeutic approach is to replace the deficient coagulation factor, which can be achieved with factors derived from human plasma or recombinants. However, despite having a therapeutic approach, most severe cases are symptomatic and may have complications, mainly in the muscles and joints. One example of such disorder is hemarthrosis. This manifestation tends to affect mainly the knee, ankle, or elbow joints in about 80% of cases. Objective: to describe the primary forms of treatment for joint bleeding in patients with severe hemophilia. Methods: This is a qualitative research of the integrative review type meant to identify productions on topics associated with hemarthrosis and severe hemophilia. The articles were searched through the databases PubMed, Scientific Electronic Library Online (Scielo) and Virtual Health Library (BVS) with the following search descriptors: “hemarthrosis andhemophilia”; “joint diseases and Hemophilia” and corresponding terms in Portuguese. The inclusion criteria were as follows:a) scientific articles b) available in full-text c) studies available in Portuguese, English, or Spanish d) randomized clinical trials e) articles published between 2016 and 2021 f) articles containing hemarthrosis caused by severe hemophilia. As exclusion criteria, texts that had no relation to the theme, did not answer the guiding question, other types of articles that did not include randomized clinical trials and/or presented duplicates were discarded. Results: In total, 42 articles were found in the selected databases; eight were duplicated, and 25 were excluded for not being randomized clinical trials or because they did not contemplate the theme. After careful reading, nine articles that met the inclusion and exclusion criteria were identified. Of the eligible studies, one reported factor replacement, and eight reported physiotherapeutic treatment. Conclusion: Factor replacement for hemophilic patients is essential and, based on the information obtained, early replacement is beneficial for the patient to avoid joint complications. Prophylaxis is indicated in severe hemophilia and its main objective is to prevent recurrent hemarthrosis, which can cause permanent functional deformities. Some physiotherapeutic interventions are indicated to prevent joint damage in severe hemophilic patients. The findings show diversity in the physical therapy modalities employed. The complete prevention of joint damage is still a challenge. A combination of treatments and a multidisciplinary team follow-up is necessary to ensure health and quality of life of patients.

Glanzmann Thrombasthenia Associated with Siderotic Synovitis and Arthropathy: A Case Report

“…It is commonly associated with chronic hemarthrosis, which involves recurrent bleeding in the joint space. 12 , 13 The pathophysiological process of hemarthrosis involves the participation of inflammatory cells, interleukins, neo-angiogenesis, and the formation of a protein complex called inflammasome, which contributes to the inflammatory response and subsequent damage to the synovial membrane. 13 This, in turn, induces synovial hypertrophy and fibrosis and, in the long run, loss of cartilage and development of subchondral cysts.…”

“…12,13 The pathophysiological process of hemarthrosis involves the participation of inflammatory cells, interleukins, neoangiogenesis, and the formation of a protein complex called inflammasome, which contributes to the inflammatory response and subsequent damage to the synovial membrane. 13 This, in turn, induces synovial hypertrophy and fibrosis and, in the long run, loss of cartilage and development of subchondral cysts. 10 There is no cure for GT, and the mainstay of management is platelet transfusion and anti-fibrinolytic agents if bleeding episodes occur or in preparation for an invasive procedure.…”