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Updated: Jun 6, 2025

A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy
Avascular necrosis in sickle cell disease needs more definitive treatment options
Najibah A Galadanci1, Julie Kanter1
1Division of Hematology and Oncology, Department of Medicine, Heersink School of Medicine, The University of Alabama at Birmingham, Birmingham, Alabama, USA.
Insights
Avascular necrosis (AVN) in sickle cell disease (SCD) causes severe pain. Patient-reported outcomes help identify better treatments for this bone condition.
Area of Science:
- Bone disease research
- Hematology
- Patient-reported outcomes
Background:
- Avascular necrosis (AVN) is bone death from disrupted blood supply.
- AVN is a common cause of severe chronic pain in sickle cell disease (SCD).
- Limited treatment options exist for AVN in SCD patients.
Purpose of the Study:
- To evaluate long-term outcomes of AVN in SCD patients.
- To assess the utility of joint-specific patient-reported outcome measures (PROMs) in studying AVN in SCD.
- To inform the development of optimal treatment strategies for AVN in SCD.
Main Methods:
- Utilized a multicentre study design.
- Employed joint-specific patient-reported outcome measures (PROMs).
- Analyzed long-term outcomes data for individuals with SCD and AVN.
Main Results:
- Patient-reported outcomes provide valuable insights into the long-term impact of AVN in SCD.
- The study highlights the potential of PROMs for treatment evaluation.
- Findings contribute to understanding the chronic pain associated with AVN in SCD.
Conclusions:
- Patient-reported outcomes are crucial for advancing the understanding and treatment of AVN in SCD.
- Further research utilizing PROMs is warranted to identify optimal therapies.
- This study moves closer to defining effective management strategies for AVN-related pain in SCD.
Abstract:
Avascular necrosis (AVN) is a bone disease caused by a temporary or definitive loss of the blood supply to bone resulting in cellular death. In people with sickle cell disease (SCD), AVN is one of the most common causes of severe chronic pain. Currently, there are very few treatment options for AVN and the current study by Casale et al. demonstrates how the use of patient-reported outcomes can bring us one step closer to identifying optimal treatments for this debilitating condition in SCD. Commentary on: Casale et al. Long-term outcomes of Avascular Necrosis in Sickle Cell Disease using joint-specific patient-reported outcome measures: Results from a multicentre study. Br J Haematol 2025; 206:310-319.
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