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Vaso-occlusive crisis as a predictor of symptomatic avascular necrosis in children with sickle cell disease
Akram Mesleh Shayeb1,2, Matthew P Smeltzer3, Sue C Kaste4,5
1Departments of Hematology, St. Jude Children's Research Hospital, Memphis, Tennessee.
Insights
Sickle cell disease (SCD) patients experiencing frequent vaso-occlusive crises (VOC) face a higher risk of developing avascular necrosis (AVN). Early radiologic screening for AVN is recommended for symptomatic individuals with over five VOC events in five years.
Area of Science:
- Hematology
- Orthopedics
- Genetics
Background:
- Avascular necrosis (AVN) is a debilitating bone complication in sickle cell disease (SCD).
- Identifying risk factors for AVN is crucial for timely screening and intervention in SCD patients.
Purpose of the Study:
- To investigate the association between vaso-occlusive crisis (VOC) frequency and the incidence of AVN in individuals with SCD.
- To identify potential risk factors for AVN in a cohort of SCD patients.
Main Methods:
- A case-control study comparing 26 symptomatic AVN cases with 128 age- and SCD genotype-matched controls.
- Data collected retrospectively between 1998 and 2014.
- Analysis focused on the frequency of VOC in the 5 years preceding AVN diagnosis.
Main Results:
- Patients experiencing 1-5 VOC events in a 5-year period showed a significantly higher likelihood of AVN (OR 11.9, P=0.02).
- Those with more than 5 VOC events in 5 years had a substantially increased risk of AVN (OR 53.6, P=0.0006).
Conclusions:
- Increased frequency of VOC is strongly associated with a higher risk of developing AVN in SCD patients.
- Symptomatic SCD patients with more than five VOC episodes in a 5-year timeframe may benefit from targeted radiologic screening for AVN.
Abstract:
Avascular necrosis (AVN) is a chronic bone complication of sickle cell disease (SCD) resulting in significant morbidity. Understanding associated risk factors can facilitate risk-based screening, earlier identification, and prompt intervention. Between 1998 and 2014, 26 symptomatic cases with imaging evidence of AVN were compared 1:5 with age- and SCD genotype-matched controls (n = 128). Patients with 1-5 vaso-occlusive crisis (VOC) (OR 11.9, 95% CI, 1.4-99.9; P = 0.02) and more than 5 VOC (OR 53.6, 95% CI, 5.5-520.2; P = 0.0006) in a 5-year period were more likely to have AVN. Symptomatic patients with more than five VOC in 5 years may benefit from radiologic screening for AVN.
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