Autoimmune myelofibrosis: A Mayo Clinic series of 22 patients.
Naseema GangatKaaren ReichardAttilio OraziAyalew TefferiPublished in: British journal of haematology (2024)
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.
Keyphrases
- end stage renal disease
- newly diagnosed
- ejection fraction
- chronic kidney disease
- primary care
- healthcare
- stem cells
- metabolic syndrome
- patient reported outcomes
- diffuse large b cell lymphoma
- skeletal muscle
- cardiac surgery
- acute kidney injury
- robot assisted
- bone marrow
- single molecule
- sickle cell disease
- hodgkin lymphoma
- smoking cessation