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Management of refractory autoimmune hemolytic anemia after allogeneic hematopoietic stem cell transplantation: current perspectives

Authors Barcellini W, Fattizzo B, Zaninoni A

Received 10 May 2019

Accepted for publication 10 July 2019

Published 8 August 2019 Volume 2019:10 Pages 265—278


Checked for plagiarism Yes

Review by Single-blind

Peer reviewer comments 2

Editor who approved publication: Dr Martin Bluth

Wilma Barcellini, Bruno Fattizzo, Anna Zaninoni

UOC Ematologia, Fondazione IRCCS Ca’ Grande Ospedale Maggiore Policlinico, Milano, Italy

Abstract: Autoimmune hemolytic anemia (AIHA) is increasingly observed after allogeneic hematopoietic stem cell transplantation (allo-HSCT), with a reported incidence between 4% and 6%. The disease is generally severe and refractory to standard therapy, with high mortality, and there are neither defined therapies, nor prospective clinical trials addressing the best treatment. Most of the knowledge on the therapy of AIHAs derives from primary forms, which are highly heterogeneous as well, further complicating the management of post-allo-HSCT forms. The review addresses the risk factors associated with post-allo-AIHA, including unrelated donor, the development of chronic extensive graft-versus-host disease, CMV reactivation, nonmalignant diagnosis pre-HSCT, and alemtuzumab use in conditioning regimens. Regarding therapy, we describe standard treatments, such as corticosteroids, intravenous immunoglobulin, splenectomy, rituximab, cyclophosphamide, and plasma exchange, which have lower response rates than those reported in primary forms. New therapeutic options, including sirolimus, bortezomib, abatacept, daratumumab and complement inhibitors, are promising tools for this detrimental complication occurring after allo-HSCT.

Keywords: autoimmune hemolytic anemia, allogeneic hematopoietic stem cell transplantation, rituximab, sirolimus and abatacept, bortezomib and daratumumab

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