Abstract
Polycythaemia rubra vera (PV) is a Philadelphia chromosome negative myeloproliferative neoplasm characterized by increased red cell production, independent of the mechanisms that regulate normal erythropoiesis. The aim of this study was to analyze the clinico-epidemiological profile of Pakistani patients with PV. In this retrospective cross sectional study, 26 patients with PV were enrolled from January 2010 to December 2014. They were diagnosed based on WHO criteria. The mean age was 53.4±9.31 years (range 36-72) and the male to female ratio was 2:1. Overall 30.7% of patients were asymptomatic. In symptomatic patients, major complaints were headache (30.8%), abdominal discomfort (23.1%), blurred vision (15.3%), pruritus (11.5%) and vascular incidents (11.5%). Physical examination revealed plethoric face and splenomegaly as predominant findings, detected in 34.6% and 30.7%, respectively, with the mean splenic span of 15.9±2.04cm. The mean hemoglobin was 18.1±1.9 g/dl with the mean hematocrit of 55.6±8.3%. The mean total leukocyte count was 12.8±7.1x109/l and the platelet count 511±341.9x109/l. Mean erythrocyte sedimentation rate was 3.5±1.22mm/hr. Serum lactate dehydrogenase, serum creatinine and uric acid were 552.7±309.2, 0.8±0.17 and 6.60±1.89 respectively. PV in Pakistani patients, unlike in the West, is seen in a moderately young population. The disease is frequently seen in male gender and primarily patients present with symptoms related to hyperviscosity.
Highlights
Myeloproliferative neoplasms (MPN) are clonal hematopoietic stem cell disorders characterized by excessive proliferation of one or more myeloid lineage cells (Sag et al, 2015; Yang et al, 2015)
Polycythaemia rubra vera (PV) is a Philadelphia chromosome negative myeloproliferative neoplasm characterized by increased red cell production, independent of the mechanisms that regulate normal erythropoiesis
Polycythaemia rubra vera belongs to clonal myeloproliferative neoplasms (MPN), characterized by unexplained red cell expansion, hyperviscosity, thromboembolism and infrequent overt bleed, could culminates into myelofibrosis (Zhang et al, 2014; Tefferi et al, 2015)
Summary
Myeloproliferative neoplasms (MPN) are clonal hematopoietic stem cell disorders characterized by excessive proliferation of one or more myeloid lineage cells (Sag et al, 2015; Yang et al, 2015). In the literature annual incidence for PV is 1-3 cases per 100,000 individuals (Sag et al, 2015; Johansson et al, 2006) It occurs most commonly in the sixth decade of life with the median age at diagnosis is ~60 years; disease may occur at any age (Thiele et al, 2008; Tefferi et al, 2013). Materials and Methods: In this retrospective cross sectional study, 26 patients with PV were enrolled from January 2010 to December 2014. They were diagnosed based on WHO criteria. The disease is frequently seen in male gender and primarily patients present with symptoms related to hyperviscosity
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