Renal Complications in Fanconi Anemia (FA) Patients Undergoing Hematopoietic Cell Transplant (HCT) Using a Uniform Radiation-Free Approach

Background

Renal and urological congenital abnormalities in patients with FA raise concern for renal dysfunction and may affect their eligibility to undergo HSCT or their post-transplant course and outcome. Some of these patients (or their physicians) may opt not to proceed with HSCT due to concerns of adverse outcome. We describe renal anomalies and associated dysfunction, and their impact on outcomes in a cohort of patients with FA undergoing HSCT.

 

Methods

A retrospective chart review of 27 patients with FA who underwent HSCT between March 2010 and May 2014.

 

Results

12 patients had structural renal/urological abnormalities at baseline.  Median nuclear GFR for these patients was 97 mL/min/1.73sq.m (range: 55-131) compared to 124 mL/min/1.73sq.m (range: 71-199) in those without renal anomalies (p=0.03). Additional patient characteristics and results are shown in Table 1. 

Post-HCT renal complications included acute kidney injury and decreased renal function measured by Cystatin C GFR.  One patient required renal replacement therapy.  Medication doses were adjusted based on Cyst C GFR for all patients.

The majority of patients (13/14) with renal impairment pre-transplant recovered to GFR>60mL/min post-transplant. 2 patients with anomalies and pre-HCT GFR <60 mL/min/1.73sq.m, are long term survivors, albeit with modest chronic kidney disease (CKD) but without the need for dialysis.

Conclusion

Patients with structural renal abnormalities had lower but surprisingly clinically acceptable pre-HCT GFR. Two patients with anomalies and pre-HCT GFR <60, are long term survivors, showing that patients with renal anomalies/marginal renal function can consider the option of HCT. Routine weekly Cystatin C-GFR allows careful and individualized medication dosing leading to improved outcomes.