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Clinico-Epidemiological Profile of Patients with Polycythaemia Rubra Vera - a Five Year Experience from a Tertiary Care Center
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 Title & Authors
Clinico-Epidemiological Profile of Patients with Polycythaemia Rubra Vera - a Five Year Experience from a Tertiary Care Center
Sultan, Sadia; Irfan, Syed Mohammed; Murad, Sania;
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 Abstract
Background: 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. 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. Results: The mean age was (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 . The mean hemoglobin was with the mean hematocrit of . The mean total leukocyte count was and the platelet count . Mean erythrocyte sedimentation rate was . Serum lactate dehydrogenase, serum creatinine and uric acid were , and respectively. Conclusions: 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.
 Keywords
Clinico-epidemiological profile;polycythaemia rubra vera;Pakistan;
 Language
English
 Cited by
 References
1.
Agarwal MB, Malhotra H, Chakrabarti P, et al (2015). Myeloproliferative neoplasms working group consensus recommendations for diagnosis and management of primary myelofibrosis, polycythaemia vera, and essential thrombocythemia. Indian J Med Paediatr Oncol, 36, 3-16. crossref(new window)

2.
Cervantes F, Passamonti F, Barosi G (2008). Life expectancy and prognostic factors in the classic BCR/ABL-negative myeloproliferative disorders. Leukemia, 22, 905-14. crossref(new window)

3.
Duangnapasatit B, Rattarittamrong E, Rattanathammethee T, et al (2015). Clinical manifestations and risk factors for complications of Philadelphia chromosome-negative myeloproliferative neoplasms. Asian Pac J Cancer Prev, 16, 5013-8. crossref(new window)

4.
Hoffbrand AV, Moss PAH. The anti-leukaemic myeloproliferative neoplasms (2011). Essential haematology, 6th edition, 208.

5.
Johansson P (2006). Epidemiology of the myeloproliferative disorders polycythaemia vera and essential thrombocythemia. Semin Thromb Hemost, 32, 171-3. crossref(new window)

6.
Khattak SA, Ahmed S, Anwar J, Bozdar M (2012). Frequency of Janus associated kinase 2 (V617F) mutation in patients of polycythemia vera. J Coll Physicians Surg Pak, 22, 80-3.

7.
Ma X, Vanasse G, Cartmel B, Wang Y, Selinger HA (2008). Prevalence of polycythaemia vera and essential thrombocythemia. Am J Hematol, 83, 359-62. crossref(new window)

8.
Sag SO, Gorukmez O, Ture M, et al (2015). MMP2 gene-735 C/T and MMP9 gene -1562 C/T polymorphisms in JAK2V617F positive myeloproliferative disorders. Asian Pac J Cancer Prev, 16, 443-9. crossref(new window)

9.
Sazawal S, Bajaj J, Chikkara S, et al (2010). Prevalence of JAK2 V617F mutation in Indian patients with chronic myeloproliferative disorders. Indian J Med Re, 132, 423-7.

10.
Sadiq MA, Ahmed S, Ali N (2013). Frequency of Janus associated kinase 2 (JAK2) mutation in patients of BCR-ABL negative myeloproliferative neoplasms. Applied Life Sciences, 2, 235-40.

11.
Tefferi A, Rumi E, Finazzi G, et al (2013). Survival and prognosis among 1545 patients with contemporary polycythaemia vera: an international study. Leukemia, 27, 1874-81. crossref(new window)

12.
Tefferi A, Barbui T (2015). Polycythaemia vera and essential thrombocythemia: 2015 update on diagnosis, risk-stratification and management. Am J Hematol, 90, 162-73. crossref(new window)

13.
Thiele J, Kvasnicka HM, Orazi A, et al (2008) . Polycythaemia vera. In: Swerdlow HS, Campo E, Haris LN, et al. WHO Classification of Tumours of Haemopoietic aAnd Lymphoid Tissues. Lyon: IARC, 40.

14.
Usman M, Bilwani F, Kakepoto GN, et al (2004). Polycythemia vera and idiopathic erythrocytosis: comparison of clinical and laboratory parameters. J Pak Med Assoc, 54, 249-51.

15.
Yang JJ, Chen H, Zheng XQ, et al (2015). Methylated alteration of SHP1 complements mutation of JAK2 tyrosine kinase in patients with myeloproliferative neoplasm. Asian Pac J Cancer Prev, 16, 2219-25. crossref(new window)

16.
Yonal-Hindilerden I, Daglar-Aday A, Akadam-Teker B, et al (2015). The burden of JAK2V617F mutated allele in Turkish patients with myeloproliferative neoplasms. J Clin Med Res, 7, 161-70. crossref(new window)

17.
Zhang ZR, Duan YC (2014). Interferon apha 2b for treating patients with JAK2V617F positive polycythaemia vera and essential thrombocytosis. Asian Pac J Cancer Prev, 15, 1681-4. crossref(new window)