Clinical and epidemiological features and therapeutic options of avascular necrosis in patients with sickle cell disease (SCD): a cross-sectional study

Main Article Content

Awni Alshurafa
Ashraf T Soliman
Vincenzo De Sanctis
Omar Ismail
Mohammad Abu-Tineh
Mohammad Khair Eddin Hemadneh
Farah Rahat Rashid
Khadra Yassin
Hana Qasim
Abdulqadir Jeprel Nashwan
Mohamed A Yassin


Sickle cell disease, avascular necrosis, osteonecrosis, hemolysis, hyperbaric oxygen


Background: Avascular necrosis (AVN) is a debilitating complication in sickle cell disease (SCD) patients, and its management is usually challenging. This study aims to evaluate the clinical and epidemiological features and therapeutic options of AVN in sickle cell patients in Qatar.

Patients and methods: A cross-sectional study was conducted on a 49 SCD patients who were diagnosed with AVN and attended the hematology clinic at the National Center for Cancer care & research, Hamad Medical Corporation, Qatar between Jan-2011 to Jan2021.

 Results: Forty-nine adult patients with SCD who were diagnosed with AVN were studied. The median age of the study population is 32 years, and the median age at the first AVN diagnosis was 26 years (range: 11-44 yr.). 37 (75.5%) patients suffered from multiple joints AVN while 12(24.5%) had single joint involvement. 31 (63.3%) patients had bilateral hip AVN and 18 (36.7%) had shoulder involvement. 30 patients (61%) were on Hydroxyurea treatment. Based on FICAT and Alert classification of AVN, 57 % of patients had stage III and above at first diagnosis. 20 (40.8%) were managed with a conservative approach, 11 (22.4%) received hyperbaric oxygen with good response, 6(12.2%) underwent hip core decompression and 12(24.5%) underwent hip replacement surgery.

Conclusion: In SCD patients, AVN occurred more during the 3rd and 4th decades of life. The majority of AVN represented with advanced stage and had multiple joint involvements. We recommend adopting a low threshold of joint imaging for early detection and prevention of further complications.


Download data is not yet available.
Abstract 186 | PDF Downloads 92


1. Khamees I, Ata F, Choudry H, Soliman AT, De Sanctis V, Yassin MA. Manifestations of HbSE sickle cell disease: a systematic review. J Transl Med. 2021; 16;19(1):262. doi: 10.1186/s12967-021-02917-w.
2. Colombatti R, Birkegård C, Medici M. PB2215: Global epidemiology of sickle cell disease: a systematic literature review. HemaSphere. 2022; 23;6(Suppl):2085-6. doi: 10.1097/01.HS9.0000762716.36058.32.
3. Abu-Shaheen A, Dahan D, Henaa H, et al. Sickle cell disease in Gulf Cooperation Council countries: a systematic review. Expert Rev Hematol. 2022;15(10):893-909. doi: 10.1080/17474086.2022.2069827.
4. Sen RK. Management of avascular necrosis of femoral head at pre-collapse stage. Indian J Orthop. 2009;43(1):6-16. doi: 10.4103/0019-5413.45323.
5. Rozi W, Rahhal A, Ali EA, et al. Direct oral anticoagulants in sickle cell disease: a systematic review and meta-analysis. Blood Adv. 2022; 13;6(17):5061-5066. doi: 10.1182/bloodadvances.2022005363.
6. Adel AA, Abushanab D, Hamad A, Al-Badriyeh D, Yassin MA. L-Glutamine and Crizanlizumab for Adults with Sickle Cell Disease (SCD) in Qatar: A Cost Effectiveness Analysis. Blood. 2021;5;138(Suppl 1):4945. doi: 10.1182/blood-2021-148232.
7. Soliman AT, Yasin M, El-Awwa A, Abdelrahman MO, De Sanctis V. Does blood transfusion affect pituitary gonadal axis and sperm parameters in young males with sickle cell disease? Indian J Endocrinol Metab. 2013;17(6):962–8. doi: 10.4103/2230-8210.122621.
8. Adesina O, Brunson A, Keegan THM, Wun T. Osteonecrosis of the femoral head in sickle cell disease: prevalence, comorbidities, and surgical outcomes in California. Blood Adv. 2017; 11;1(16):1287–95. doi: 10.1182/bloodadvances.2017006117.
9. Al Fadhali I, Al-Kindy F, Alshibli N, et al. Prevalence and Outcome of Avascular Necrosis of the Hip (AVN) Among Young Omani Patients with Sickle Cell Disease. Blood. 2019; 13;134(Suppl 1):3577. doi: 10.1182/blood-2019-130436.
10. Elalfy MS, Fadhli I, Mohammad S, et al. Avascular Necrosis of the Femoral Head in Sickle Cell Disease in Egypt and Oman: A Cross Sectional Study. Blood. 2018 ; 29;132(Suppl 1):4921. doi: 10.1182/blood-2018-99-112624.
11. Milner PF, Kraus AP, Sebes JI, et al. Sickle cell disease as a cause of osteonecrosis of the femoral head. N Engl J Med. 1991; 21;325(21):1476–81. doi: 10.1056/NEJM199111213252104.
12. Yawn BP, Buchanan GR, Afenyi-Annan AN, et al. Management of sickle cell disease: summary of the 2014 evidence-based report by expert panel members. JAMA. 2014 10;312(10):1033–48. doi: 10.1001/jama.2014.10517.
13. Vezzani G, Quartesan S, Cancellara P, et al. Hyperbaric oxygen therapy modulates serum OPG/RANKL in femoral head necrosis patients. J Enzyme Inhib Med Chem. 2017 ;32(1):707–11. doi: 10.1080/14756366.2017.1338447.
14. Bosco G, Vezzani G, Mrakic Sposta S, et al. Hyperbaric oxygen therapy ameliorates osteonecrosis in patients by modulating inflammation and oxidative stress. J Enzyme Inhib Med Chem. 2018 ;33(1):1501–5. doi: 10.1080/14756366.2018.1505693..
15. Camporesi EM, Vezzani G, Bosco G, Mangar D, Bernasek TL. Hyperbaric oxygen therapy in femoral head necrosis. J Arthroplasty. 2010 Sep;25(6 Suppl):118–23. doi: 10.1016/j.arth.2010.05.010.
16. Alshurafa A, Elhissi M, Yassin MA. Complete resolution of stage II avascular necrosis affecting three joints by hyperbaric oxygen in a patient with sickle cell disease: A case report. Front Med. 2022;9:1063255. doi: 10.3389/fmed.2022.1063255.
17. Shier A, Abdelrazek M, Soliman A, et al. Short-Term Outcome and MRI Changes in Three Adult Patients with Sickle Cell Disease and Aseptic Osteonecrosis after Treatment with Hyperbaric Oxygen Therapy: A Preliminary Report. Case Rep Oncol. 2020;13(1):365–72. doi: 10.1159/000507429.

Most read articles by the same author(s)

1 2 3 4 5 6 7 8 9 10 11 12 > >>