What is the success rate of kidney transplant in patients with sickle cell anemia (SCA) and end-stage renal disease (ESRD)?

Kidney Transplant Success Rates in Sickle Cell Anemia

Kidney transplantation in patients with sickle cell disease and end-stage renal disease achieves approximately 88% one-year patient survival and 85% one-year graft survival, with outcomes comparable to diabetic nephropathy patients, making transplantation the preferred treatment over long-term dialysis despite higher mortality compared to the general transplant population. 1, 2

Short-Term Outcomes (1 Year)

• Patient survival at 1 year post-transplant is 88% (95% CI, 80.1-95.5) based on pooled data from 6 observational studies involving 307 SCD patients 1, 2
• Graft survival at 1 year is 85% for cadaveric transplants in SCD patients 1
• These one-year outcomes are reasonably strong, though SCD patients face approximately twice the mortality risk compared to the general transplant population (HR 2.03; 95% CI, 1.31-3.16) 1

Long-Term Outcomes (Beyond 1 Year)

The evidence becomes more variable and concerning after the first year:

• Graft survival at 3 years drops to 48% in one study, which was lower than the 60% rate in non-SCD ESRD patients (P = 0.055) 1
• However, a contradictory study showed comparable graft survival at 2,5, and 10 years between SCD and non-SCD patients 1
• Graft rejection occurs in approximately 23% of SCD transplant recipients across multiple studies 1

Critical Comparison: Transplant vs. Dialysis

The survival benefit of transplantation over dialysis is substantial and represents the primary justification for proceeding with transplant 1:

• SCD patients who undergo transplant show a trend toward improved survival versus those remaining on dialysis (relative risk 0.14; P < 0.056) 1
• Recent research confirms similar mortality reduction with transplantation in SCD patients compared to other ESRD etiologies, with an absolute risk difference of 20.3 percentage points at 10 years 3
• Transplant outcomes in SCD are comparable to diabetic nephropathy patients (SCD 73.1% vs. diabetes 74.1%; P = 0.44) 1

Disease-Specific Complications Post-Transplant

Sickle cell pain crises remain common after transplantation, occurring in 64% of patients 1, likely exacerbated by:

• Corticosteroid use in immunosuppression regimens 1
• Ongoing sickling physiology despite improved renal function 1
• Decline in renal function can occur following transplant 1

Evidence-Based Recommendation Framework

The American Society of Hematology recommends proceeding with kidney transplantation over long-term dialysis for SCD patients with ESRD, despite very low certainty of evidence, based on these key drivers 1:

1. ESRD occurs at earlier ages in SCD than other conditions, making the burden of lifelong dialysis particularly severe 1
2. Survival outcomes are improving in more recent cohorts, suggesting continued advancement in transplant techniques 1
3. Dialysis outcomes remain poor for adults with SCD, making transplantation the better option despite its risks 1

Perioperative Management Considerations

Strict adherence to perioperative transfusion protocols is essential 1, 2:

• Follow general SCD guidelines for perioperative transfusion requirements 2
• Automated exchange blood transfusion programs may improve outcomes, with one study showing superior patient survival, graft survival, and graft function in those receiving exchange transfusions versus those who did not 4
• Use corticosteroids judiciously in immunosuppression regimens due to their association with vaso-occlusive pain 1

Common Pitfalls to Avoid

• Do not deny transplant referral based on SCD diagnosis alone: Despite lower survival compared to the general transplant population, the survival benefit over dialysis is substantial and comparable to other high-risk groups like diabetic patients 1, 3
• Recognize access disparities: SCD patients are less likely to receive transplantation even after waitlist registration (subdistribution HR 0.62; 95% CI, 0.53-0.72), representing a significant equity issue 3
• Monitor for recurrent sickle cell nephropathy in the transplanted kidney, which can cause permanent decline in graft function 5
• Anticipate higher infection rates, particularly cytomegalovirus and bacterial infections, in the post-transplant period 6

Quality of Life Considerations

Kidney transplantation offers additional years of life and better quality of life compared to dialysis 2, avoiding the substantial burden of thrice-weekly dialysis sessions while providing the opportunity for improved hemoglobin levels post-transplant 1.