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Posterior reversible encephalopathy syndrome (PRES) after haploidentical haematopoietic stem cell transplantation: incidence, risk factors and outcomes

Abstract

Posterior reversible encephalopathy syndrome (PRES) is a gradually recognised neurological complication of allogenic haematopoietic stem cell transplantation (allo-HSCT). However, there is a paucity of information on PRES after haploidentical HSCT (haplo-HSCT). We performed a retrospective nested case-control study in patients following haplo-HSCT for malignant and nonmalignant haematologic diseases between January 2009 and December 2018 in our centre. A total of 45 patients were diagnosed with PRES after transplant, accounting for an incidence of 1.17%. Grades II to IV acute graft-versus-host disease (aGVHD) (HR 2.370, 95% CI 1.277–4.397, p = 0.006) and hypertension (HR 14.466, 95% CI 7.107–29.443, p < 0.001) were identified as risk factors for developing PRES after haplo-HSCT. There was no difference in overall survival (OS), disease-free survival (DFS), the cumulative incidence of relapse or nonrelapse mortality (NRM) between patients with PRES and controls without PRES following haplo-HSCT in either adults or children. All but one patient with PRES showed nearly complete clinical and neurologic recovery. In conclusion, PRES is a rare condition with benign outcomes following haplo-HSCT. Further multicentre prospective studies are needed to confirm the results and help to establish the standard therapy for posttransplant PRES.

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Fig. 1: Imaging manifestations of PRES.
Fig. 2: Clinical outcomes of patients with PRES after haplo-HSCT.

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Acknowledgements

The work was supported by the Beijing Municipal Science and Technology Commission (No. Z171100001017084), the Beijing Natural Science Foundation (H2018206423), the Key Program of the National Natural Science Foundation of China (No. 81730004), the Foundation for Innovative Research Groups of the National Natural Science Foundation of China (81621001), the Beijing Natural Science Foundation (No. 7171013), National Natural Science Foundation of China (No. 81670116) and the National Key Research and Development Program of China (No. 2017YFA0105500, No. 2017YFA0105503).

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Chen, Q., Zhao, X., Fu, HX. et al. Posterior reversible encephalopathy syndrome (PRES) after haploidentical haematopoietic stem cell transplantation: incidence, risk factors and outcomes. Bone Marrow Transplant 55, 2035–2042 (2020). https://doi.org/10.1038/s41409-020-0894-5

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