No abstract

Abdi and colleagues reported important insights regarding the treatment history of non-severe hemophilia A (HA) patients, 1 including incidence of joint bleeds in a large, international, and multi-center non-severe HA cohort (the INSIGHT cohort). Repeated intraarticular bleeding episodes in hemophilia patients may lead to degenerative arthropathy, which is the most frequent complication in HA regardless of the degree of severity. Despite the interesting findings described on annualized bleeding rate in mild-to-moderate HA patients, the study by Abdi and colleagues did not provide information on the joint status. The early identification of joint damage is indeed crucial to prevent the progression to hemophilic arthropathy, and thus periodic monitorization of the joint status is recommended for non-severe HA patients. 2 However, subclinical intra-articular bleeds may be hardly noticeable by clinical examination, especially in milder forms of HA; hemarthrosis and abnormalities may not be present for many years after birth, joint health awareness is usually lower, and most clinical evidence is generally extrapolated from severe patients' data. 3Diagnostic imaging is necessary to identify subtle alterations, and MRI is the gold standard for joint evaluation. Nevertheless, access to this technique is limited and its low feasibility for regular or on-demand (OD) check-ups has led to the implementation of musculoskeletal ultrasound as a point-of-care technique, with several reports supporting its usefulness. 4 This has been associated with the development of Hemophilia Early Arthropathy Detection with Ultrasound (HEAD-US), 5 a scoring system which enables a low-cost and quick evaluation of the six major joints, namely left and right sided elbows (LE, RE), knees (LK, RK), and ankles (LA, RA). Here we report unpublished findings of a 28-adult male patient subset with moderate (n = 14) and mild (n = 14) HA, with median age of 42.5 [IQR 32-60.5] years (40.5 [IQR 30.0-49.0] and 51.0 [IQR 34.0-63.0] years for moderate and mild, respectively) from a previously performed cross-sectional study to assess joint status in Spain. 6Not surprisingly we found 19 patients non-subjected to any evaluation by imaging modalities over the last 12 months (85.7%

No abstract

Total knee arthroplasty (TKA) is a commonly used option in advanced stages of knee arthropathy in people with hemophilia (PWH). The objective of this article is to determine what the complication rates and implant survival rates in PWH are in the literature. A literature search was carried out in PubMed (MEDLINE), Cochrane Library, Web of Science, Embase and Google Scholar utilizing the keywords “hemophilia TKA complications” on 20 October 2022. It was found that the rate of complications after TKA in PWH is high (range 7% to 30%), although it has improved during the last two decades, possibly due to better perioperative hematologic treatment. However, prosthetic survival at 10 years has not changed substantially, being in the last 30 years approximately 80% to 90% taking as endpoint the revision for any reason. Survival at 20 years taking as endpoint the revision for any reason is 60%. It is possible that with a precise perioperative control of hemostasis in PWH, the percentage of complications after TKA can be diminished.

Background: It is important to discard those practices that do not add value. As a result, several initiatives have emerged. All of them try to improve patient safety and the use of health resources. Purpose: Present a compendium of "do not do recommendations" in the context of hemophilia. Methods: A review of the literature and current clinical guidelines has been made, based on the best evidence available to date. Results: The following 13 recommendations stand out: 1) Do not delay the administration of factor after trauma; 2) do not use fresh frozen plasma or cryoprecipitate; 3) do not use desmopressin in case of hematuria; 4) do not change the product in the first 50 prophylaxis exposures; 5) do not interrupt immunotolerance; 6) do not administer aspirin or NSAIDs; 7) do not administer intramuscular injections; 8) do not do routine radiographs of the joint in case of acute hemarthrosis; 9) Do not apply closed casts for fractures; 10) do not discourage the performance of physical activities; 11) do not deny surgery to a patient with an inhibitor; 12) not perform instrumental deliveries in fetuses with hemophilia; 13) do not use factor IX (FIX) in patients with hemophilia B with inhibitor and a history of anaphylaxis after administration of FIX. Conclusions: The information mentioned previously can be useful in the management of hemophilia, from different levels of care. As far as we know, this is the first initiative of this type in hemophilia.