Indication and management of allogeneic haematopoietic stem-cell transplantation in myelofibrosis: updated recommendations by the EBMT/ELN International Working Group

Nicolaus Kröger, Andrea Bacigalupo, Tiziano Barbui, Markus Ditschkowski, Nico Gagelmann, Martin Griesshammer, Vikas Gupta, Nada Hamad, Claire Harrison, Juan Carlos Hernandez-Boluda, Steffen Koschmieder, Tania Jain, John Mascarenhas, Ruben Mesa, Uday R. Popat, Francesco Passamonti, Nicola Polverelli, Alessandro Rambaldi, Marie Robin, Rachel B. SalitThomas Schroeder, Bart L. Scott, Roni Tamari, Ayalew Tefferi, Alessandro M. Vannucchi, Donal P. McLornan, Giovanni Barosi

Research output: Contribution to journalReview articlepeer-review

6 Scopus citations

Abstract

New options for medical therapy and risk scoring systems containing molecular data are leading to increased complexity in the management of patients with myelofibrosis. To inform patients’ optimal care, we updated the 2015 guidelines on indications for and management of allogeneic haematopoietic stem-cell transplantation (HSCT) with the support of the European Society for Blood and Marrow Transplantation (EBMT) and European LeukemiaNet (ELN). New recommendations were produced using a consensus-building methodology after a comprehensive review of articles released from January, 2015 to December, 2022. Seven domains and 18 key questions were selected through a series of questionnaires using a Delphi process. Key recommendations in this update include: patients with primary myelofibrosis and an intermediate-2 or high-risk Dynamic International Prognostic Scoring System score, or a high-risk Mutation-Enhanced International Prognostic Score Systems (MIPSS70 or MIPSS70-plus) score, or a low-risk or intermediate-risk Myelofibrosis Transplant Scoring System score should be considered candidates for allogeneic HSCT. All patients who are candidates for allogeneic HSCT with splenomegaly greater than 5 cm below the left costal margin or splenomegaly-related symptoms should receive a spleen-directed treatment, ideally with a JAK-inhibitor; HLA-matched sibling donors remain the preferred donor source to date. Reduced intensity conditioning and myeloablative conditioning are both valid options for patients with myelofibrosis. Regular post-transplantation driver mutation monitoring is recommended to detect and treat early relapse with donor lymphocyte infusion. In a disease where evidence-based guidance is scarce, these recommendations might help clinicians and patients in shared decision making.

Original languageEnglish
Pages (from-to)e62-e74
JournalThe Lancet Haematology
Volume11
Issue number1
DOIs
StatePublished - Jan 2024

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