E-ISSN: 1308-5263
Impact of the HEAD-US Scoring System for Observing the Protective Effect of Prophylaxis in Hemophilia Patients: A Prospective, Multicenter, Observational Study [Turk J Hematol]
Turk J Hematol. 2021; 38(2): 101-110 | DOI: 10.4274/tjh.galenos.2021.2020.0717  

Impact of the HEAD-US Scoring System for Observing the Protective Effect of Prophylaxis in Hemophilia Patients: A Prospective, Multicenter, Observational Study

Kaan Kavaklı1, Süha Süreyya Özbek2, Ali Bülent Antmen3, Fahri Şahin4, Şevkiye Selin Aytaç5, Alphan Küpesiz6, Bülent Zülfikar7, Mehmet Sönmez8, Ümran Çalışkan9, Can Balkan1, Tuğana Akbaş10, Taner Arpacı10, İpek Tamsel2, Turgut Seber11, Berna Oğuz11, Can Çevikol12, Mesut Bulakçı7, Polat Koşucu13, Demet Aydoğdu14, İlgen Şaşmaz3, Gülen Tüysüz6, Başak Koç7, Hüseyin Tokgöz9, Zuhal Demirci4, Burcu Özkan15
1Ege University Children’s Hospital, Clinic of Children’s Health and Diseases, Division of Pediatric Hematology, İzmir, Turkey
2Ege University Medical Faculty Hospital, Clinic of Radiology, Division of Hematology, İzmir, Turkey
3Acıbadem Adana Hospital, Clinic of Pediatric Hematology, Adana, Turkey
4Ege University Medical Faculty Hospital, Clinic of Internal Diseases, Division of Hematology, İzmir, Turkey
5Hacettepe University Faculty of Medicine, Department of Children’s Health and Diseases, Division of Pediatric Hematology, Ankara, Turkey
6Akdeniz University Hospital, Clinic of Children’s Health and Diseases, Antalya, Turkey
7İstanbul University Hemophilia Comprehensive Care Center, İstanbul, Turkey
8Karadeniz Technical University Medical Faculty Farabi Hospital, Clinic of Internal Diseases, Division of Hematology, Trabzon, Turkey
9Necmettin Erbakan University Meram Medical Faculty Hospital, Department of Children’s Health and Diseases, Konya, Turkey
10Acıbadem Adana Hospital, Clinic of Radiology, Adana, Turkey
11Hacettepe University Faculty of Medicine, Department of Radiology, Ankara, Turkey
12Akdeniz University Hospital, Clinic of Radiology, Antalya, Turkey
13Karadeniz Technical University Medical Faculty Farabi Hospital, Clinic of Radiology, Trabzon, Turkey
14Necmettin Erbakan University Meram Medical Faculty Hospital, Clinic of Radiology, Konya, Turkey
15Pfizer Pharmaceuticals, Rare Disease Department, İstanbul, Turkey

Objective: This study aimed to observe the preventive effect of prophylactic treatment on joint health in people with hemophilia (PwH) and to investigate the importance of integration of ultrasonographic examination into clinical and radiological evaluation of the joints.
Materials and Methods: This national, multicenter, prospective, observational study included male patients aged ≥6 years with the diagnosis of moderate or severe hemophilia A or B from 8 centers across Turkey between January 2017 and March 2019. Patients were followed for 1 year with 5 visits (baseline and 3rd, 6th, 9th, and 12th month visits). The Hemophilia Joint Health Score (HJHS) was used for physical examination of joints, the Pettersson scoring system was used for radiological assessment, point-of-care (POC) ultrasonography was used for bilateral examinations of joints, and the Hemophilia Early Arthropathy Detection with Ultrasound (HEAD-US) score was used for evaluation of ultrasonography results.
Results: Seventy-three PwH, of whom 62 had hemophilia A and 11 had hemophilia B, were included and 24.7% had target joints at baseline. The HJHS and HEAD-US scores were significantly increased at the 12th month in all patients. These scores were also higher in the hemophilia 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 the HJHS total score and Pettersson total score at baseline and at the 12th month.
Conclusion: The HEAD-US and HJHS scoring systems are valuable tools during follow-up examinations of PwH and they complement each other. We suggest that POC ultrasonographic evaluation and the HEAD-US scoring system may be integrated into differential diagnosis of bleeding and long-term monitoring for joint health as a routine procedure.

Keywords: Hemophilic arthropathy, Joint Scores, HJHS, Ultrasonography


Hemofili Hastalarında Profilaksinin Koruyucu Etkisinin Gözlenmesinde HEAD-US Skorlama Sisteminin Önemi: Prospektif, Çok Merkezli ve Gözlemsel Bir Çalışma

Kaan Kavaklı1, Süha Süreyya Özbek2, Ali Bülent Antmen3, Fahri Şahin4, Şevkiye Selin Aytaç5, Alphan Küpesiz6, Bülent Zülfikar7, Mehmet Sönmez8, Ümran Çalışkan9, Can Balkan1, Tuğana Akbaş10, Taner Arpacı10, İpek Tamsel2, Turgut Seber11, Berna Oğuz11, Can Çevikol12, Mesut Bulakçı7, Polat Koşucu13, Demet Aydoğdu14, İlgen Şaşmaz3, Gülen Tüysüz6, Başak Koç7, Hüseyin Tokgöz9, Zuhal Demirci4, Burcu Özkan15
1Ege University Children’s Hospital, Clinic of Children’s Health and Diseases, Division of Pediatric Hematology, İzmir, Turkey
2Ege University Medical Faculty Hospital, Clinic of Radiology, Division of Hematology, İzmir, Turkey
3Acıbadem Adana Hospital, Clinic of Pediatric Hematology, Adana, Turkey
4Ege University Medical Faculty Hospital, Clinic of Internal Diseases, Division of Hematology, İzmir, Turkey
5Hacettepe University Faculty of Medicine, Department of Children’s Health and Diseases, Division of Pediatric Hematology, Ankara, Turkey
6Akdeniz University Hospital, Clinic of Children’s Health and Diseases, Antalya, Turkey
7İstanbul University Hemophilia Comprehensive Care Center, İstanbul, Turkey
8Karadeniz Technical University Medical Faculty Farabi Hospital, Clinic of Internal Diseases, Division of Hematology, Trabzon, Turkey
9Necmettin Erbakan University Meram Medical Faculty Hospital, Department of Children’s Health and Diseases, Konya, Turkey
10Acıbadem Adana Hospital, Clinic of Radiology, Adana, Turkey
11Hacettepe University Faculty of Medicine, Department of Radiology, Ankara, Turkey
12Akdeniz University Hospital, Clinic of Radiology, Antalya, Turkey
13Karadeniz Technical University Medical Faculty Farabi Hospital, Clinic of Radiology, Trabzon, Turkey
14Necmettin Erbakan University Meram Medical Faculty Hospital, Clinic of Radiology, Konya, Turkey
15Pfizer Pharmaceuticals, Rare Disease Department, İstanbul, Turkey

Amaç: Bu çalışmada hemofili hastalarında profilaksinin eklem sağlığı üzerindeki koruyucu etkisini gözlemlemek ve eklemlerin klinik ve ultrasonografik olarak değerlendirilmesinin öneminin araştırılması amaçlandı.
Gereç ve Yöntem: Ulusal, çok merkezli, prospektif ve gözlemsel olan araştırma Ocak 2017 - Mart 2019 arasında 8 ayrı merkezde takip edilen erkek ve 6 yaşından büyük orta seviyede veya ağır hemofili-A ve hemofili-B hastalarını kapsıyordu. Hastalar 1 yıllık takip sırasında toplam 5 ayrı vizitte (başlangıç, 3. ay, 6. ay, 9. ay ve 12. ay) değerlendirildi. Hemofili Eklem Sağlığı Skoru (HJHS) eklemlerin fiziksel bakısında kullanılırken Petterson skorlama sistemi radyolojik bakı sırasında değerlendirildi. Ayrıca, hasta başı ultrasonografisiyle bilateral eklem incelemeleri yapılarak HEAD-US skorlama yöntemiyle eklem skorları belirlendi.
Bulgular: Çalışmada 62’si hemofili A ve 11’I hemofili B olan 73 hastanın %24,7’sinde hedef eklem varlığı mevcuttu. HJHS ve HEAD-US skorlarının tüm hastalarda takibin 12. ayında anlamlı olarak arttığı gözlendi. Hemofili A hastalarında daha yüksek skorlar saptandı. Skor artımı alt gruplarda değerlendirildiğinde çocukluk yaş grubunda artışın anlamlı olmadığı gözlendi. Başlangıçta ve 12. ayda yapılan üçlü bakıda HJHS, HEAD-US ve Petterson skorlarının anlamlı olarak korele olduğu saptandı.
Sonuç: HJHS eklem skoru ile HEAD-US radyolojik skorlamasının hemofili hastalarının eklem sağlığının takibinde çok değerli olup birbirlerini destekledikleri yakından gözlendi. Hasta başı US skorlama sistemlerinin günümüzde hemofilide eklem sağlığının rutin takip ve izlemi sürecinde hem kanama ayırıcı tanısı hem de uzun dönemli takip açısından oldukça değerli bir yeri olacağını düşünüyoruz.

Anahtar Kelimeler: Hemofilik artropati, Eklem skorları, HJHS, Ultrasonografi


Kaan Kavaklı, Süha Süreyya Özbek, Ali Bülent Antmen, Fahri Şahin, Şevkiye Selin Aytaç, Alphan Küpesiz, Bülent Zülfikar, Mehmet Sönmez, Ümran Çalışkan, Can Balkan, Tuğana Akbaş, Taner Arpacı, İpek Tamsel, Turgut Seber, Berna Oğuz, Can Çevikol, Mesut Bulakçı, Polat Koşucu, Demet Aydoğdu, İlgen Şaşmaz, Gülen Tüysüz, Başak Koç, Hüseyin Tokgöz, Zuhal Demirci, Burcu Özkan. Impact of the HEAD-US Scoring System for Observing the Protective Effect of Prophylaxis in Hemophilia Patients: A Prospective, Multicenter, Observational Study. Turk J Hematol. 2021; 38(2): 101-110

Corresponding Author: Kaan Kavaklı, Türkiye


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Impact Factor (2019) = 1.685