Hemophilic arthropathy (HA) patients frequently have perioperative pain after total knee arthroplasty (TKA). Although periarticular local infiltration analgesia by using a cocktail has been utilized in various surgeries, the efficacy of cocktail administration in HA patients undergoing TKA remains unclear. This study aims to determine whether cocktail therapy can relieve perioperative pain and improve postoperative rehabilitation activities after TKA in HA patients, and whether the addition of corticosteroids to the cocktail is both effective and necessary. We conducted a retrospective analysis of clinical data from 98 HA patients who underwent TKA at our institution between January 2015 and January 2024. All surgeries were performed by two senior orthopedic surgeons and two assistants from our team, using posterior-stabilized prostheses. The patients were divided into two groups: the experimental group (ropivacaine 100 mg + morphine 10 mg + dexamethasone 35 mg + normal saline 50 ml, n = 45) and the control group (ropivacaine 100 mg + morphine 10 mg + normal saline 50 ml, n = 53). A three-month follow-up study was conducted to compare the postoperative outcomes in the above groups, including Visual Analogue Scale (VAS) scores, knee range of motion (ROM), Knee Society Score (KSS), inflammatory markers (C-reactive protein, CRP, and Interleukin- 6, IL- 6), and hospitalization parameters (body temperature, length of hospital stay, hospitalization costs, and perioperative usage of coagulation factor VIII). Both groups improved knee joint function and reduced post-operative pain at the last follow-up. With knee ROM increasing from 47.22° to 95.36° and KSS increasing from 35.42 to 82.02, the experimental group's VAS ratings dropped from 4.31 to 1.47. Besides, the control group's VAS scores dropped from 4.71 to 2.09, knee ROM increased from 45.95° to 91.60°, and KSS from 36.87 to 80.40 (all with P < 0.05). Throughout the follow-up, the experimental group showed better pain reduction (P < 0.05), with greater knee ROM and KSS within the first month compared to the control group. This difference diminished by the third month, but the experimental group still showed higher knee ROM and KSS. Additionally, the experimental group exhibited lower inflammation markers and a shorter hospital stay (P < 0.05). In people with hemophilia undergoing TKA the cocktail of ropivacaine 100 mg + morphine 10 mg + nomal saline 50 mL + 35 mg dexamethasone seemed to be more effective in relieving postoperative pain than the cocktail of ropivacaine 100 mg + morphine 10 mg + nomal saline 50 mL at 3-month follow-up.

Total hip arthroplasty (THA) effectively treats end-stage hemophilic hip arthropathy. Given hemophilia's unique characteristics, perioperative bleeding remains a significant risk for patients undergoing THA. Tranexamic acid (TXA), an efficient antifibrinolytic agent, may benefit the outcomes of THA for patients with hemophilia (PWH). This study aims to explore the clinical efficacy of intra-articular injection of TXA in treating perioperative bleeding in PWH and assess its additional clinical benefits.

The retrospective study comprised data of PWH who received THA from January 2015 to December 2021 in the research center. A total of 59 individuals were included in the study, divided into a TXA group (n = 31) and a non-TXA group (n = 28). We compared various parameters, including total blood loss (TBL), visible blood loss (VBL), occult blood loss (OBL), intraoperative coagulation factor VIII (FVIII) consumption, perioperative total FVIII consumption, hemoglobin (HB), erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), length of hospital stay, hospitalization costs, length of surgery, total protein, activated partial thromboplastin time (APTT), D-dimer, rate of joint swelling, hip joint range of motion (ROM), visual analogue scale (VAS), and Harris hip joint function scale (HHS) between the two groups. Follow-up assessments were conducted for up to 24 months. A Student's t test was utilized for the statistical analysis.

This study demonstrated that intra-articular TXA effectively reduced TBL (1248.19 ± 439.88 mL, p < 0.001), VBL (490.32 ± 344.34 mL, p = 0.003), and OBL (757.87 ± 381.48 mL, p = 0.004) in PWH who underwent THA. TXA demonstrated effectiveness in reducing VAS scores on POD1, POD7, and POD14 and joint swelling rates on POD1, POD7, POD14, and at discharge (p < 0.05). Additionally, the TXA group achieved higher HHS ratings at all follow-up time points (p < 0.05), showing superior hip joint mobility, lower postoperative inflammation levels, reduced factor VIII consumption during surgery, and less postoperative nutritional loss. No statistically significant differences were observed between the two groups in terms of hospital stay, hospitalization costs, surgery duration, and coagulation indicators.

Intra-articular injection of TXA reduces perioperative bleeding in PWH undergoing THA while also improving joint mobility, post-operative rehabilitation, and quality of life. This may provide value for the future application of TXA in PWH.

Transformational advances have occurred in the management of haemophilia in the last decade leading to much better outcomes. However, a detailed and critical examination of its assessment and reporting show gaps in many aspects. These are discussed in this review.

The relevant literature related to different aspects of management of haemophilia was reviewed to identify gaps which need to be addressed. These include detection and diagnosis of haemophilia, documentation and reporting of joint bleeding, its management and methods of reporting in clinical trials and practice, aspects of personalizing care as well as access to therapeutic products and the need for and organization of comprehensive care.

Current diagnostic approaches have more than doubled the identified number of persons with haemophilia (PWH) over the last 25 years but still constitute only ∼30% of the expected number. Joint bleeding is the primary indicator of disease severity and treatment efficacy, but there is lack of consistency and standardization in the way it is recorded and reported. Its continued use as an efficacy measure of modern treatments which maintain steady state factor levels or equivalence of >5% will lack sensitivity. The treatment of acute haemarthrosis has focussed on haemostasis and pain control, ignoring the role of inflammation in joint damage. Phenotypic heterogeneity of severe haemophilia has recognized clinical and laboratory variations based on haemostasis but not differences in local response to blood in the joint. At the organizational level, IU/capita provides a relevant measure of access to therapeutic products when the detection rate is ∼100% but is fallaciously low when detection rates are very low. With highly effective modern therapies for haemophilia and nearly no bleeding, the concept of comprehensive care team will need modifications.

As haemophilia care advances, a deeper dive is needed into the details of various aspects its management to ensure consistency and contemporary relevance.

Previous studies have reported marked interindividual variation in factor VIII (FVIII) clearance in patients with hemophilia (PWH) and proposed a number of factors that influence this heterogeneity.

To investigate the importance of the clearance rates of endogenous von Willebrand factor (VWF) compared with those of other FVIII half-life modifiers in adult PWH.

The half-life of recombinant FVIII was determined in a cohort of 61 adult PWH. A range of reported modifiers of FVIII clearance was assessed (including plasma VWF:antigen and VWF propeptide levels; VWF-FVIII binding capacity; ABO blood group; and nonneutralizing anti-FVIII antibodies). The FVIII-binding region of the VWF gene was sequenced. Finally, the effects of variation in FVIII half-life on clinical phenotype were investigated.

We demonstrated that heterogeneity in the clearance of endogenous plasma VWF is a key determinant of variable FVIII half-life in PWH. Both ABO blood group and age significantly impact FVIII clearance. The effect of ABO blood group on FVIII half-life in PWH is modulated entirely through its effect on the clearance rates of endogenous VWF. In contrast, the age-related effect on FVIII clearance is, at least in part, VWF independent. In contrast to previous studies, no major effects of variation in VWF-FVIII binding affinity on FVIII clearance were observed. Although high-titer immunoglobulin G antibodies (≥1:80) were observed in 26% of PWH, these did not impact FVIII half-life. Importantly, the annual FVIII usage (IU/kg/y) was significantly (p = .0035) increased in patients with an FVIII half-life of <12 hours.

Our data demonstrate that heterogeneity in the half-life of FVIII concentrates in patients with hemophilia A is primarily attributable to variability in the clearance of endogenous VWF.