Incidence and Risk Factors of Pain Crisis After Hematopoietic Cell Transplantation for Sickle Cell Disease

Vaso-occlusive episodes (VOC) or pain crises are the commonest indications for hematopoietic cell transplantation (HCT) for sickle cell disease (SCD). Elimination of pain crisis post-HCT is an important patient-centered outcome and may improve understanding of the natural history of pain syndromes in SCD. We examined de-identified records of 763 patients followed for a median of 36.7 months (0.3-168.6) with 69.6% patient’s age at HCT <18 years, 83.3% patient’s Karnofsky-Lansky Performance (KPS) score ≥ 90, overall survival 92.9%, event-free survival 72.4%, graft failure (GF) 22.4%, AGVHD 21.4%, CGVHD 27%, and pain crisis 8.65%. On unadjusted logistic regression, increased risk of pain crisis post-HCT was observed in patients with age at HCT >10 years (11-17 years [OR=9.43; 95% CI: 3.20, 27.79, p<0.0001], age ≥ 18 years [ OR=16.62; 95% CI: 5.85, 47.16, p<0.0001]), history of pain crisis 2 years before HCT [OR= 13.16, 95% CI: 4.08, 42.42, p<0.0001], alternate donors (haploidentical [OR= 4.80; 95% CI: 2.48, 9.31, p<0.0001], unrelated matched [OR= 2.71; 95% CI: 1.23, 5.97, p=0.0132], and mismatched unrelated [OR= 3.19; 95% CI: 1.44, 7.05, p=0.0041], and with GF [n= 41(5.37%), OR = 7.15; 95% CI: 4.20, 12.18., p<0.0001]. Pain crisis was less frequent with KPS ≥90 [OR = 0.31; 95% CI: 0.18, 0.55, p<0.0001]. Multivariable logistic regression models confirmed age at HCT, KPS, graft type, donor type, history of VOC 2 yearspre-HCT, and GF as independent predictors of pain crisis post-HCT and generated predictive models and nomograms for pain crisis post-HCT for SCD which can support shared decision-making.