Primary Idiopathic Myelofibrosis: Clinico-Epidemiological Profile and Risk Stratification in Pakistani Patients

Background: Primary idiopathic myelofibrosis (PMF) is a clonal Philadelphia chromosome-negative myeloproliferative neoplasm characterized by extramedullary hematopoiesis and marrow fibrosis. It is an uncommon hematopoietic malignancy which primarily affects elderly individuals. The rational of this study was to determine its clinico-epidemiological profile along with risk stratification in Pakistani patients. Materials and
Methods: In this retrospective cross sectional study, 20 patients with idiopathic myelofibrosis were enrolled from January 2011 to December 2014. Data were analyzed with SPSS version 22.
Results: The mean age was 57.9±16.5 years with 70% of patients aged above 50. The male to female ratio was 3:1. Overall only 10% of patients were asymptomatic and the remainder presented with constitutional symptoms. In symptomatic patients, major complaints were weakness (80%), weight loss (75%), abdominal discomfort (60%), night sweats (13%), pruritus (5%) and cardiovascular accidents (5%). Physical examination revealed splenomegaly as a predominant finding detected in 17 patients (85%) with the mean splenic span of 22.2±2.04cm. The mean hemoglobin was 9.16±2.52 g/dl with the mean MCV of 88.2±19.7 fl. The total leukocyte count of 17.6 19.2x109/l and platelets count were 346.5±321.9x109/l. Serum lactate dehydrogenase, serum creatinine and uric acid were 731.0±154.1, 0.82±0.22 and 4.76±1.33 respectively. According to risk stratification, 35% were in high risk, 40% in intermediate risk and 25% in low risk groups.
Conclusions: The majority of PMF patients were male and presented with constitutional symptoms in our setting. Risk stratification revealed predominance of advanced disease in our series.