Clinico-Pathological Features and Outcome of Primary Myelofibrosis: Experience from a Developing Country

Aqsa Ali Khattak; Mehreen Ali khan; Mehwish Gilani; Nabeela Khan; Saima Humayun; Yasir Abbas

Authors

Aqsa Ali Khattak Department of Clinical Haematology and Bone Marrow Transplant, National University of Medical Sciences, Rawalpindi
Mehreen Ali khan Department of Clinical Haematology and Bone Marrow Transplant , National University of Medical Sciences, Rawalpindi, Pakistan
Mehwish Gilani Department of Clinical Haematology and Bone Marrow Transplant , National University of Medical Sciences, Rawalpindi, Pakistan
Nabeela Khan Department of Clinical Haematology and Bone Marrow Transplant , National University of Medical Sciences, Rawalpindi, Pakistan
Saima Humayun Department of Clinical Haematology and Bone Marrow Transplant , National University of Medical Sciences, Rawalpindi, Pakistan
Yasir Abbas Department of Clinical Haematology and Bone Marrow Transplant , National University of Medical Sciences, Rawalpindi, Pakistan

Keywords:

Primary Myelofibrosis

Abstract

Objective The objective of the study is to assess clinical and laboratory parameters in PMF patients presenting to our institute.

Methodology: This prospective observational study was carried out at Armed Forces Bone Marrow Transplant Center from October 2022 to March 2025. The sample size was calculated with a 95% confidence level, resulting in a total of 210 patients. All patients, irrespective of gender were included. Patients were diagnosed with MF (pre-fibrotic/Overt fibrotic stage) based on history, clinical examination and WHO diagnostic criteria 2022. All samples were screened for mutations in JAK 2 V617F, JAK 2 Exon12, CALR and, cMPL. Data analysis was done using SPSS version 23.

Results: A total of 210 patients were included in the study, with the mean age of 53.1 ± 16.6 years. There were 70% (n=147) males and 30% (n=63) females. Most the patients i.e. 71.0% (n=149) were diagnosed with overt phase of PMF, while 15.2% of the patients presented in pre-fibrotic phase of PMF. The common symptoms at presentation was weakness and easy fatigability and progressive pallor. On examination around 66.2% patients presented with splenomegaly and 33.8% had hepatomegaly. As per DIPPS scoring system 20% (n=42) were having low risk disease, while 33%, 37% and 9% fell under intermediate 1, intermediate 2 and high risk disease respectively. A total of 25 patients (11.9%) experienced disease transformation during the study period. The overall survival (OS) of patients diagnosed with PMF in our cohort is 65.6%, with a median follow-up of 36 months, and Progression-free survival (PFS) is calculate to be 84.4%.

Conclusion: This study offers real-world data on primary myelofibrosis (PMF) from a developing country and underscores the significant gaps in clinical care. This study also highlights a low risk of transformation in MPNs in our population. Further prospective studies are required to understand the full clinical spectrum of the disease better and to compare the outcomes of different treatment approaches.