Autoimmune myelofibrosis: A Mayo Clinic series of 22 patients

Naseema Gangat; Kaaren Reichard; Attilio Orazi; Ayalew Tefferi

doi:10.1111/bjh.19499

Autoimmune myelofibrosis: A Mayo Clinic series of 22 patients

Br J Haematol. 2024 May 2. doi: 10.1111/bjh.19499. Online ahead of print.

Authors

Naseema Gangat¹, Kaaren Reichard², Attilio Orazi³, Ayalew Tefferi¹

Affiliations

¹ Division of Hematology, Mayo Clinic, Rochester, Minnesota, USA.
² Division of Hematopathology, Mayo Clinic, Rochester, Minnesota, USA.
³ Department of Pathology, Texas Tech University Health Sciences Center, El Paso, Texas, USA.

PMID: 38698680
DOI: 10.1111/bjh.19499

Abstract

We describe the clinical phenotype, management strategies and outcomes of 22 patients with autoimmune myelofibrosis (AIMF); median age: 45 years; 77% females; 83% with autoimmune disease, pancytopenia in 32% and transfusion-requiring anaemia in 59%. All informative cases were negative for JAK2 (n = 18) and CALR/MPL mutations (n = 12). Fourteen of nineteen (74%) evaluable patients achieved complete response (CR) based on the resolution of cytopenias. First-line treatments included steroids +/- immunosuppressive agents, cyclosporin and mycophenolate with CR in 7 of 13 (54%), 1 of 2 (50%) and 1 of 2 (50%) respectively. Rituximab salvage therapy yielded CR in 4 of 5 (80%) cases. The current study provides information on steroid-sparing treatments for AIMF.

Keywords: JAK2; autoimmune; fibrosis; myeloproliferative.