Good treatment-free survival of monoclonal gammopathy of undetermined significance associated pure red cell aplasia after bortezomib plus dexamethasone

Blood Cells Mol Dis. 2021 Jul:89:102573. doi: 10.1016/j.bcmd.2021.102573. Epub 2021 Apr 27.

Abstract

Pure red cell aplasia (PRCA) is a rare syndrome characterized by severe anemia and absence of erythroid precursors. PRCA associated to monoclonal gammopathy of undetermined significance (MGUS) is a scarce condition with less than five cases reported so far. There is no agreement on the treatment of MGUS associated PRCA and treatment- free survival (TFS) is an unmet clinical need. In this report, for the first time, we demonstrated two patients with MGUS associated PRCA obtained rapid remission and maintained TFS after accepting intensive short-term bortezomib plus dexamethasone. The first case was refractory to cyclosporine and prednisone, but achieved complete remission after ten doses of bortezomib. Moreover, he has kept TFS for 12 months. The other case initiated bortezomib plus dexamethasone as soon as making a definite diagnosis. She obtained complete remission after twelve doses of bortezomib and she has maintained a normal level of haemoglobin for 8 months.

Keywords: Bortezomib; Monoclonal gammopathy of undetermined significance; Pure red cell aplasia; Treatment free survival.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Antineoplastic Agents / therapeutic use*
  • Bortezomib / therapeutic use*
  • Dexamethasone / therapeutic use*
  • Female
  • Humans
  • Male
  • Middle Aged
  • Monoclonal Gammopathy of Undetermined Significance / complications*
  • Red-Cell Aplasia, Pure / drug therapy*
  • Red-Cell Aplasia, Pure / etiology*
  • Survival Analysis

Substances

  • Antineoplastic Agents
  • Bortezomib
  • Dexamethasone