Results from a retrospective study suggest that identification of patients with primary myelofibrosis likely to survive 20+ years can be made on the basis of age, sex, blood counts, and symptoms, without inclusion of genetic risk factors. This study was published in the American Journal of Hematology.
Primary myelofibrosis is a chronic blood cancer associated with the presence of scar tissue in the bone marrow, and impairments in the production of normal blood cells. Patients with primary myelofibrosis have a median survival of 6 years, and the only treatment shown to alter the natural history of the disease is allogeneic hematopoietic stem cell transplantation (allo-HSCT). However, allo-HSCT is associated with the need for immunosuppression, and has associated risks of infection, graft-vs-host disease, and treatment-related death. Hence, careful selection of potential candidates for early allo-HSCT is critically important. Accordingly, there are a number of existing risk models that facilitate treatment selection in primary myelofibrosis. The main objective of this study was to identify phenotypic and genotypic characteristics of long-lived patients with primary myelofibrosis.
A total of 1282 consecutive patients with primary myelofibrosis were retrospectively identified in the Mayo Clinic database between April 1976 and November 2017. Of these patients, 26 (2%) survived 20+ years from the time of diagnosis, and 626 (49%) died within the first 5 years following diagnosis; these 2 groups were compared. Multivariate analysis identified 7 independent predictors of long-term survival which included age 70 years or younger (P =.002), female sex (P = .03), hemoglobin level 10 g/dL or higher for women and 11 g/dL or higher for men (P =.03), leukocyte count less than 25 × 109/L (P =.009), platelet count 100 × 109/L or higher (P=.002), circulating blasts 1% or less (P =.03), and absence of constitutional symptoms (P =.04). Interestingly, genetic risk factors were not shown to be independent predictors of long-term survival in primary myelofibrosis.
“This study provides complementary information, and it is not necessarily a competing risk model, that facilitates prognostic information, in addition to what is provided from current prognostic models,” the authors clarified.
Penna D, Lasho TL, Finke CM, et al. 20+ Years and alive with primary myelofibrosis: Phenotypic signature of very long-lived patients. Am J Hematol. 2019;94(3):286-290.