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Bone mineral density in children with fanconi anemia after hematopoietic cell transplantation
Anna Petryk1, Lynda E Polgreen2, Jessie L Barnum3
1Department of Pediatrics, Division of Pediatric Endocrinology, University of Minnesota, Minneapolis, Minnesota.
Insights
Children with Fanconi anemia (FA) undergoing hematopoietic cell transplantation (HCT) show reduced bone mineral density (BMD) compared to healthy peers. Monitoring BMD is crucial for bone health in FA patients post-HCT.
Area of Science:
- Pediatric Hematology
- Endocrinology
- Genetics
Background:
- Fanconi anemia (FA) is an inherited DNA repair disorder causing bone marrow failure.
- Hematopoietic cell transplantation (HCT) is a common treatment for FA.
- Bone mineral density (BMD) changes after HCT are known in leukemia but understudied in FA.
Purpose of the Study:
- Compare BMD in FA patients post-HCT to healthy controls and cancer patients post-HCT.
- Identify risk factors associated with bone loss in FA patients after HCT.
Main Methods:
- Cross-sectional study of 20 FA HCT, 13 cancer HCT, and 90 healthy controls (<18 years).
- Dual-energy x-ray absorptiometry (DXA) measured total body (TBMD) and lumbar spine (LBMD) Z-scores.
- BMD Z-scores adjusted for height-for-age Z-score (HAZ).
Main Results:
- FA HCT patients had significantly lower mean TBMDHAZ Z-scores (-0.8 SD) than healthy controls.
- 42% of FA HCT patients had TBMD Z-scores ≤ -1, versus 11% of controls.
- No significant LBMD deficits were found; FA HCT and cancer HCT groups showed similar BMD.
Conclusions:
- FA patients post-HCT exhibit reduced total body BMD compared to healthy children.
- Lower body mass index and body fat percentage correlate with lower BMD in FA HCT patients.
- Regular BMD monitoring is recommended for FA patients to ensure optimal bone health.
Abstract:
Fanconi anemia (FA) is an inherited DNA repair disorder associated with short stature and bone marrow failure, usually requiring hematopoietic cell transplantation (HCT). Although low bone mineral density (BMD) has been reported in leukemia patients after HCT, little is known about BMD in FA children after HCT (FA HCT). This study's goals were to compare BMD in FA HCT to BMD in healthy controls and in children who received HCT for hematologic malignancy (cancer HCT), and to test for associations between BMD and risk factors for bone loss. This cross-sectional study included 20 FA HCT, 13 cancer HCT, and 90 healthy controls, age-matched and <18 years old at evaluation. BMD Z-scores for total body (TBMD) and lumbar spine (LBMD) were measured by dual energy x-ray absorptiometry and adjusted for height-for-age Z-score (HAZ). FA HCT had lower mean TBMDHAZ Z-score (by .8 SD) and higher fraction with Z-score ≤ -1 than healthy controls (42% versus 11%). No LBMD deficits were detected. FA HCT and cancer HCT groups did not differ significantly in TBMD or LBMD Z-scores. In FA HCT patients, lower body mass index and lower percent fat were associated with lower BMD. This study highlights the importance of monitoring BMD to optimize bone health in FA patients.
