MYELOFIBROSIS PROGRESSION is often incessant and not always apparent1,2
Understanding risk and features of MF
of patients will have intermediate-2 or high-risk disease at diagnosis3
~1.3 and ~2.9 years median OS for high-risk and intermediate-2 MF, respectively4,5
Signs and symptoms of MF6,7
- MF typically presents with splenomegaly, impaired blood cell production, and constitutional symptoms, including fatigue, night sweats, itching, fever, and weight loss
MF progression leads to extensive bone marrow fibrosis (BMF)—a key feature of the disease8
Consequences of BMF: Higher-grade BMF correlates with poor survival1,9-12
- Extensive BMF contributes to impaired hematopoiesis and clinical features including, but not limited to, splenomegaly8-10,13
- A retrospective study showed that patients with a higher-grade BMF had an ~3-fold decrease in median survival vs patients with a BMF of ≤1 (P<0.001)11
Higher grades of BMF were associated with clinical manifestations of MF, including8:
Onset of
ANEMIA
Higher percentage of
BLASTS IN THE PERIPHERAL BLOOD
Increased
SPLEEN SIZE
The consequences of myelofibrosis progression continue to impact patients’ lives1
JAK/STAT=Janus kinase signal transducer and activator of transcription; MF=myelofibrosis; OS=overall survival.
References: 1. Kramann R, Schneider RK. The identification of fibrosis-driving myofibroblast precursors reveals new therapeutic avenues in myelofibrosis. Blood. 2018;131(19):2111-2119. doi:10.1182/blood-2018-02-834820 2. Palandri F, Al-Ali HK, Guglielmelli P, et al. Impact of bone marrow fibrosis grade on response and outcomes in patients with primary myelofibrosis treated with ruxolitinib: a post-hoc analysis of the JUMP study. Poster EP1092 presented at: EHA2021; June 9-17, 2021; Virtual Congress. 3. Tefferi A, Lasho TL, Jimma T, et al. One thousand patients with primary myelofibrosis: the Mayo Clinic experience. Mayo Clin Proc. 2012;87(1):25-33. doi:10.1016/j.mayocp.2011.11.001 4. Pettit K, Odenike O. Novel therapies for myelofibrosis. Curr Hematol Malig Rep. 2017;12(6):611-624. doi:10.1007/s11899-017-0403-0 5. Gangat N, Caramazza D, Vaidya R, et al. DIPSS Plus: a refined Dynamic International Prognostic Scoring System for primary myelofibrosis that incorporates prognostic information from karyotype, platelet count, and transfusion status. J Clin Oncol. 2011;29(4):392-397. doi:10.1200/JCO.2010.32.2446 6. Reilly JT, McMullin MF, Beer PA, et al. Guideline for the diagnosis and management of myelofibrosis. Br J Hematol. 2012;158(4):453-471. doi:10.1111/j.1365-2141.2012.09179 7. Mesa RA, Miller CB, Thyne M, et al. Myeloproliferative neoplasms (MPNs) have a significant impact on patients’ overall health and productivity: the MPN Landmark survey. BMC Cancer. 2016;16:167. doi:10.1186/s12885-016-2208-2 8. Zahr AA, Salama ME, Carreau N, et al. Bone marrow fibrosis in myelofibrosis: pathogenesis, prognosis and targeted strategies. Haematologica. 2016;101(6):660-671. doi:10.3324/haematol.2015.141283 9. Gleitz HFE, Kramann R, Schneider RK. Understanding deregulated cellular and molecular dynamics in the haematopoietic stem cell niche to develop novel therapeutics for bone marrow fibrosis. J Pathol. 2018;245(2):138-146. doi:10.1002/path.5078 10. Agarwal A, Morrone K, Bartenstein M, Zhao ZJ, Verma A, Goel S. Bone marrow fibrosis in primary myelofibrosis: pathogenic mechanisms and the role of TGF-β. Stem Cell lnvestig. 2016;3(5):1-10. doi:10.3978/j.issn.2306-9759.2016.02.03 11. Lekovic D, Gotic M, Perunicic-Jovanovic M, et al. Contribution of comorbidities and grade of bone marrow fibrosis to the prognosis of survival in patients with primary myelofibrosis. Med Oncol. 2014;31(3):869. doi:10.1007/s12032-014-0869-8 12. Gianelli U, Vener C, Bossi A, et al. The European Consensus on grading of bone marrow fibrosis allows a better prognostication of patients with primary myelofibrosis. Mod Pathol. 2012;25(9):1193-1202. doi:10.1038/modpathol.2012.87 13. Gleitz HFE, Pritchard JE, Kramann R, Schneider RK. Fibrosis driving myofibroblast precursors in MPN and new therapeutic pathways. HemaSphere. 2019;3(S2):142-145. doi:10.1097/HS9.0000000000000216
ABBV-DE-00894-MC