Allogeneic hematopoietic cell transplantation is an effective treatment for patients with Richter syndrome: A systematic review and meta-analysis

Hematol Oncol Stem Cell Ther. 2021 Mar;14(1):33-40. doi: 10.1016/j.hemonc.2020.05.002. Epub 2020 May 15.

Abstract

Efficacy of conventional chemoimmunotherapy is limited in patients with Richter syndrome (RS) with anticipated median overall survival (OS) of less than 10 months. Allogeneic hematopoietic cell transplantation (allo-HCT) is commonly offered as a consolidative treatment option in RS. To our knowledge, there are no randomized controlled studies that have compared allo-HCT against other therapies in RS; available allo-HCT data are limited to small case series from single-institution or registry studies. We performed a systematic review and meta-analysis to assess the totality of evidence regarding the efficacy (or lack thereof) of allo-HCT for RS. We extracted data on post-allograft outcomes related to benefits (overall response rate [ORR], complete remission [CR], OS, and progression-free survival [PFS]). For harms, data were extracted on non-relapse mortality (NRM) and relapse post-allografting. Our search strategy identified 240 studies, but only four studies (n = 72 patients) met our inclusion criteria. Pooled ORR, CR, OS, and PFS rates were 79%, 33%, 49%, and 30%, respectively. Pooled NRM and relapse rates were 24% and 28%, respectively. Results of this systematic review and meta-analysis indicate that allo-HCT yields encouraging OS in RS, thus remaining a reasonable treatment option in fit patients whose disease demonstrates a chemosensitive response to pre-transplant salvage therapies. Novel strategies are certainly needed to reduce the risk of relapse to further improve outcomes in these patients.

Keywords: Allogeneic hematopoietic cell transplantation; Overall survival; Richter syndrome.

Publication types

  • Meta-Analysis
  • Systematic Review

MeSH terms

  • Allografts
  • Disease-Free Survival
  • Hematopoietic Stem Cell Transplantation*
  • Humans
  • Leukemia, Lymphocytic, Chronic, B-Cell* / complications
  • Leukemia, Lymphocytic, Chronic, B-Cell* / mortality
  • Leukemia, Lymphocytic, Chronic, B-Cell* / therapy
  • Lymphoma, B-Cell* / etiology
  • Lymphoma, B-Cell* / mortality
  • Lymphoma, B-Cell* / therapy
  • Registries*
  • Survival Rate
  • Syndrome