A pilot study of orelabrutinib treatment in three cases of refractory/relapsed autoimmune haemolytic anaemia/Evans syndrome

Br J Haematol. 2024 Mar;204(3):1082-1085. doi: 10.1111/bjh.19146. Epub 2023 Nov 6.

Abstract

Currently, there is no effective treatment for refractory/relapsed (R/R) autoimmune haemolytic anaemia (AIHA), associated with poor quality of life. Bruton tyrosine kinase inhibitors have begun to be used in some autoimmune diseases. We initiated the clinical trial of orelabrutinib treatment on R/R AIHA/Evans Syndrome, which is in progress. The preliminary results showed that nine of the 12 enrolled patients responded to orelabrutinib treatment. Here, we reported three cases who have completed the treatment and were followed up for 6 months, achieving complete or partial remission. Orelabrutinib is expected to become a new second-line treatment for R/R AIHA/Evans syndrome.

Keywords: Evans syndrome; autoimmune haemolytic anaemia; orelabrutinib; refractory; relapsed.

MeSH terms

  • Anemia, Hemolytic, Autoimmune* / therapy
  • Humans
  • Pilot Projects
  • Piperidines*
  • Pyridines*
  • Quality of Life
  • Thrombocytopenia*

Substances

  • orelabrutinib
  • Piperidines
  • Pyridines

Supplementary concepts

  • Evans Syndrome