Interferon Apha 2b for Treating Patients with JAK2V617F Positive Polycythemia Vera and Essential Thrombocytosis

Zhang, Zhirong; Duan, Ying

doi:10.7314/apjcp.2014.15.4.1681

Cited by 9 publications

(19 citation statements)

References 15 publications

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“…Polycythaemia vera belongs to clonal myeloproliferative neoplasms, exemplified by increased red cell proliferation, hyperviscosity, thromboembolism and intermittently overt bleed (Zhang et al, 2014;Tefferi et al, 2015). The present study detected the somatic JAK2 V617F mutation in 92.3% of Pakistani patients with PV.…”

Section: Discussionsupporting

confidence: 47%

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Somatic JAK-2 V617F Mutational Analysis in Polycythemia Rubra Vera: a Tertiary Care Center Experience

Sultan

Irfan²,

Khan³

2016

Asian Pacific Journal of Cancer Prevention

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Background: Polycythemia rubra vera (PV), being a primary polycythemia, is caused by neoplastic proliferation of erythroid, megakaryocytic and granulocytic lineages which result in panmyelosis. PV patients have a somatic acquired mutation in the Janus kinase (JAK2) pathway, rendering cell proliferation independent of the normal regulatory mechanisms that regulate erythropoiesis. The rational of this study was to determine the prevalence of the JAK-2 V617F mutation in Pakistani patients with PV. Materials and Methods: In this cross sectional study, 26 patients with PV were enrolled from January 2010 to December 2014. Patients were diagnosed based on WHO criteria for PV. All were screened for G-T point mutation (V617F) in the JAK2 gene on chromosome 9 by an allele specific PCR. Results: The mean age was 53.4±9.31 years (range 36-72) and the male to female ratio was 2:1. The frequency of JAK2 V617F positivity in our PV patients was found to be 92.3%. Overall 30.7% of patients were asymptomatic and remaining 69.3% presented with symptomatic disease. The mean hemoglobin was 18.1±1.9g/dl with the mean hematocrit of 55.6±8.3%. The mean total leukocyte count was 12.8±7.1x10 9 /l and the platelet count was 511±341.9x10 9 /l. A positive correlation of JAK2 V617F mutation was established with high TLC count (P=0.01). No correlation of JAK2 V617F could be established with age or gender (P>0.05). Conclusions: The JAK2 V617F mutation frequency in our PV patients was similar to those reported internationally. Screening for the mutation in all suspected PV cases could be beneficial in differentiating patients with reactive and clonal erythrocytosis.

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Section: Discussionsupporting

confidence: 47%

“…Polycythaemia rubra vera (PV) is a indolent MPN characterized by an absolute increase in the number of red blood cells, usually accompanied by leukocytosis, thrombocytosis and splenomegaly (Ma et al, 2008;Zhang et al, 2014).…”

Section: Introductionmentioning

confidence: 99%

Somatic JAK-2 V617F Mutational Analysis in Polycythemia Rubra Vera: a Tertiary Care Center Experience

Sultan

Irfan²,

Khan³

2016

Asian Pacific Journal of Cancer Prevention

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“…Essential thrombocythemia (ET) belongs to clonal myeloproliferative neoplasms (MPN), characterized by unexplained platelets augmentation, overt thromboembolism, clinical bleeding and infrequently could culminates into myelofibrosis or leukemia (Zhang et al, 2014). It is an acquired, Philadelphia chromosome (Ph)-negative, indolent rare malignancy of hemopoeitic stem cells (Zhang et al, 2014).…”

Section: Discussionmentioning

confidence: 99%

“…It is an acquired, Philadelphia chromosome (Ph)-negative, indolent rare malignancy of hemopoeitic stem cells (Zhang et al, 2014).…”

Section: Discussionmentioning

confidence: 99%

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Clinico-Hematological Profile and Risk Stratification in Patients with Essential Thrombocythemia: Experience from Pakistan

Sultan

Irfan²,

Tanveer³

et al. 2015

Asian Pacific Journal of Cancer Prevention

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Background: Essential thrombocythemia (ET) is a Philadelphia chromosome-negative myeloproliferative neoplasm characterized by sustained thrombocytosis and megakaryocytic hyperplasia. It is an uncommon hematological malignancy which primarily affects elderly individuals. The rational of this study was to determine its clinico-hematological profile along with risk stratification in Pakistan patients. Materials and Methods: In this retrospective cross sectional study, 21 patients with ET were enrolled from January 2011 to December 2014. Data was analyzed with SPSS version 21. Results: The mean age was 56.7±19.0 years (range 18-87) and the male to female ratio was 1:1.1. Of the total, 62% of patients were above 50 years of age. Overall 61.9% were diagnosed incidentally and were asymptomatic. In symptomatic patients, major complaints were weakness (19%); erythromelalgia (14.2%), transit ischemic attack (9.5%) and gastrointestinal bleed (4.7%). The mean hemoglobin count was 11.7±2.4 g/dl with a total leukocyte count of 13.3±8.1x10 9 /l and platelets count of 1188.8±522.2x10 9 /l. Serum lactate dehydrogenase, serum creatinine and uric acid were 454.3±127.8, 1.2±0.5 and 7.4±3.4 respectively. According to risk stratification, 57.1% were in high risk; 23.8% in intermediate risk while 19.1% in low risk group. Conclusions: ET in our patients in Pakistan, unlike in the West, is seen in a relatively young population. Primarily patients were asymptomatic and risk stratification revealed predominance of high risk disease in our setting.

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How We Identify and Manage Patients with Inadequately Controlled Polycythemia Vera

Reiter

Harrison

2016

Curr Hematol Malig Rep

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Polycythemia vera (PV) is a chronic myeloproliferative neoplasm (MPN) characterized by an overactive Janus kinase/signal transducer and activator of transcription (JAK/STAT) pathway through mutations in JAK2 exons 12 or 14 (JAK2 V617F). The dominant clinical characteristics include erythrocytosis (with or without leukocytosis/thrombocytosis), thrombotic events, and symptoms. Increased risk of mortality is mainly caused by thrombotic events and progression to post-polycythemia vera myelofibrosis (PPV-MF) or secondary acute myeloid leukemia (sAML). The most important prognostic factors include age and a history of thrombotic events, although recent evidence has indicated that leukocytosis and additional cytogenetic aberrations may also be of significant prognostic value. First-line therapies include aspirin and phlebotomies, which significantly reduce the incidence of thrombotic events and prolong survival. Cytoreductive treatment with hydroxyurea (approved) and conventional or pegylated interferon-α (effective, but not approved in many countries) is initiated for high-risk or inadequately controlled disease, e.g., uncontrolled hematocrit, leukocytosis, thrombocytosis, thrombotic events, splenomegaly, or symptoms. However, some patients may not receive initial benefit from first-line therapy or may become resistant or intolerant in due course. Although second-line treatment options are limited, clinical trials have shown the efficacy of ruxolitinib toward improving blood counts, enlarged spleen, and symptoms and potentially reducing thrombotic events. Identification of patients with uncontrolled PV is important for clinical care, as such patients have a high risk of complications, and future studies with JAK inhibitors or other agents alone or in combination are needed to test their potential to reduce rates of thrombotic events and transformation to PPV-MF or sAML.

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Interferon Apha 2b for Treating Patients with JAK2V617F Positive Polycythemia Vera and Essential Thrombocytosis

Cited by 9 publications

References 15 publications

Somatic JAK-2 V617F Mutational Analysis in Polycythemia Rubra Vera: a Tertiary Care Center Experience

Somatic JAK-2 V617F Mutational Analysis in Polycythemia Rubra Vera: a Tertiary Care Center Experience

Clinico-Hematological Profile and Risk Stratification in Patients with Essential Thrombocythemia: Experience from Pakistan

How We Identify and Manage Patients with Inadequately Controlled Polycythemia Vera

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