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Kavaklı K. et al: Ultrasound and Monitoring for HemophiliaTurk J Hematol 2021;38:101-110AbstractResults: Seventy-three PwH, of whom 62 had hemophilia A and11 had hemophilia B, were included and 24.7% had target joints atbaseline. The HJHS and HEAD-US scores were significantly increasedat the 12 th month in all patients. These scores were also higher in thehemophilia A subgroup than the hemophilia B subgroup. However,in the childhood group, the increment of scores was not significant.The HEAD-US total score was significantly correlated with both theHJHS total score and Pettersson total score at baseline and at the 12 thmonth.Conclusion: The HEAD-US and HJHS scoring systems are valuabletools during follow-up examinations of PwH and they complementeach other. We suggest that POC ultrasonographic evaluation and theHEAD-US scoring system may be integrated into differential diagnosisof bleeding and long-term monitoring for joint health as a routineprocedure.Keywords: Hemophilic arthropathy, Joint Scores, HJHS, UltrasonographyÖzBulgular: Çalışmada 62’si hemofili A ve 11’I hemofili B olan 73 hastanın%24,7’sinde hedef eklem varlığı mevcuttu. HJHS ve HEAD-US skorlarınıntüm hastalarda takibin 12. ayında anlamlı olarak arttığı gözlendi. HemofiliA hastalarında daha yüksek skorlar saptandı. Skor artımı alt gruplardadeğerlendirildiğinde çocukluk yaş grubunda artışın anlamlı olmadığıgözlendi. Başlangıçta ve 12. ayda yapılan üçlü bakıda HJHS, HEAD-US vePetterson skorlarının anlamlı olarak korele olduğu saptandı.Sonuç: HJHS eklem skoru ile HEAD-US radyolojik skorlamasının hemofilihastalarının eklem sağlığının takibinde çok değerli olup birbirlerinidestekledikleri yakından gözlendi. Hasta başı US skorlama sistemleriningünümüzde hemofilide eklem sağlığının rutin takip ve izlemi sürecindehem kanama ayırıcı tanısı hem de uzun dönemli takip açısından oldukçadeğerli bir yeri olacağını düşünüyoruz.Anahtar Sözcükler: Hemofilik artropati, Eklem skorları, HJHS, UltrasonografiIntroductionThe goal of hemophilia treatment is to prevent bleeding byreplacement of factor concentrates or substitution of missingcoagulation factors. Coagulation factors can be given forprophylaxis (primary and secondary prophylaxis, etc.) orfor treatment when needed [1]. Prevention of bleeding viaprophylaxis is considered the gold-standard treatment in casesof severe hemophilia [1,2]. Provision of early prophylaxis forseverely hemophilic children can completely or largely preventlife-threatening bleeding, chronic joint diseases, and disabilities;thus, requirements for surgical interventions can be decreasedand both health and social well-being of people with hemophilia(PwH) can be improved [1,2,3].Evaluation of joint status is crucial not only for staging jointdisease but also for the follow-up of prophylaxis and forevaluating outcomes of replacement therapy. Joint functionis widely assessed by the Hemophilia Joint Health Score(HJHS), but implementation of the HJHS requires training andexperience. The Pettersson scoring system, a radiological jointscoring system, gives quite reliable outcomes when applied byan experienced radiologist [4,5]. Magnetic resonance imaging(MRI) is a more sensitive imaging method than plain radiogramin evaluating the joints. Nevertheless, MRI has disadvantagessuch as long scanning period, high cost, limited availability,and need for sedation in young children [4,5]. However,ultrasonography may provide advantages such as appropriatecost, availability, repeatability, faster examination, no need forsedation while examining children, and scanning of multiplejoints and dynamic examination of joints in a single session.The present study aimed to observe the preventive effectof prophylactic treatment on joint health in PwH and toinvestigate the importance of integration of point of-care (POC)ultrasonographic examination into clinical and radiologicalevaluation of the joints.Materials and MethodsPatientsThe current study was designed as a national, multicenter,prospective, non-interventional, observational study. A total of 8centers across Turkey were selected and the data were collectedfrom January 2017 through March 2019. Male patients aged≥6 years with the diagnosis of moderate or severe hemophilia Aor hemophilia B (factor level <2%) were included in the study.The pediatric group (47.9%) ranged in age from 6 to 18 yearswhile the adult group (52.1%) ranged in age from 19 to 70 years(Table 1).Patients with communication difficulties (unable to understandor speak Turkish) or cognitive dysfunction and patients withinhibitors were excluded. The study was approved by the ClinicalResearch Ethics Committee of the Ege University Faculty ofMedicine and written informed consent was obtained from thepatients or their legal representatives.ProcedureAfter enrollment in the study, the patients were followed for12 months with a total of 5 visits (baseline and 3 rd , 6 th , 9 th ,and 12 th months). In the study centers, patient data concerningdemographic features and hemophilia history were recorded oncase report forms at the baseline visit. Physical examinationsof elbow, knee, and ankle joints were performed with the HJHSduring each visit. Annual bleeding rate (ABR) was recordedevery 3 months during clinical visits.102
Turk J Hematol 2021;38:101-110Kavaklı K. et al: Ultrasound and Monitoring for HemophiliaAdditionally, ultrasonographic examinations of the bilateralelbow, ankle, and knee joints were performed during each visit.Ultrasonography results of the patients were evaluated withthe Hemophilia Early Arthropathy Detection with Ultrasound(HEAD-US) scoring system. Evaluations were performed byexpert physiotherapists and radiologists. The same experts ineach center performed the evaluations at different time pointsto prevent variability.For all patients, quality of life (QoL) questionnaires wereadministered at the baseline, 6 th month, and 12 th month visits.Compliance of prophylaxis and bleeding episodes were recordedevery 3 months in clinical visits.MeasurementsThe HJHS is a scoring system used to assess physical joint damagein PwH and recommended for routine follow-up assessments ofjoint health. Using the HJHS, the six most commonly involvedjoints (elbows, knees, and ankles) are evaluated in PwH andtotal scores are within the range of 0-124. High scores indicatedamage/impairment [6].The Pettersson scoring system allows detailed evaluation ofradiological changes in the joints. Posterior-anterior and lateralX-ray images of the joints are evaluated. Scores range from 0 to13 for a single joint and the maximum possible total score is 78when 6 joints are evaluated [7,8].Table 1. Demographics of 73 patients receiving prophylaxis.Group Children Adults AllPatients, n 35 38 73(%) (47.9%) (52.1%) (100%)Ages (years)Range 6-17 18-70 6-70Median 14 36 18Mean ± SD 11.3±6.2 39.6±26.4 22.1±14.5Body mass indexRange 13.2-24.8 24.1-35.2 13.2-35.2Median 15.9 28.6 23.8Mean ± SD 16.1±5.6 25.6±14.2 23.4±5.2Type of hemophilia All patients Hemophilia A Hemophilia Bn 73 62 11Ages (years)Range 6-70 12-60Median 17.5 19.0Mean ± SD 21.3±14.1 26.5±16.7SeveritySevere 68 58 10Moderate 5 4 1*Presence of target joint 18 (24.3%) 15 3*Orthopedic operation history 23 (31.5%) 20 3*Radioisotope synovectomy 6 (8.2%) 5 1Joint bleedingKnee-right 15 13 2Knee-left 15 13 2Elbow-right 18 15 3Elbow-left 14 12 2Ankle-right 20 17 3Ankle-left 14 13 1All patients had severe HA or HB.SD: Standard deviation.N103
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Turk J Hematol 2021;38:155-174LETTE
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Advisory Board of This Issue (June
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