Methylated Alteration of SHP1 Complements Mutation of JAK2 Tyrosine Kinase in Patients with Myeloproliferative Neoplasm

Yang, Junjun; Chen, Hui; Zheng, Xiaoqun; Li, Haiying; Wu, Jianbo; Tang, Liyuan; Gao, Shenmeng

doi:10.7314/apjcp.2015.16.6.2219

Cited by 10 publications

(12 citation statements)

References 31 publications

(24 reference statements)

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Section: Discussionmentioning

confidence: 99%

“…Thus, demethylation therapy that targets SHP1 (which is involved in regulating the JAK2/STAT signalling pathway) may contribute to treatment for patients with myeloproliferative neoplasm. 11 Investigation of methylation using genome arrays has revealed that gene hypermethylation is a promotive factor for PMF transforming to AML. 12,13 Thepot et al 6 found that the overall response rate (ORR) in patients with myeloproliferative neoplasm transformed to AML, who were treated with azacytidine, was 38%, with a median overall survival of 8 months.…”

Section: Discussionmentioning

confidence: 99%

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Decitabine combined with all-trans retinoic acid as treatment in a case of primary myelofibrosis transforming into acute myeloid leukaemia

et al. 2019

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Primary myelofibrosis (PMF) is a type of cloned myeloproliferative neoplasm stemming from haematopoietic stem cells, and tends to transform to acute myeloid leukaemia (AML) in approximately 10–20% of cases over a 10-year period. The transformation into AML has a poor prognosis, with a median overall survival of only 2.6 months in patients receiving supportive treatment. To date, treatment of AML transformation remains poor. The case of a 58-year-old female patient with AML transformed from PMF, who was treated with decitabine combined with all-trans retinoic acid, is reported. The patient had complete remission and a 17-month overall survival from initial diagnosis of transformed AML, with tolerated haematologic toxicity during the treatment period.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Decitabine combined with all-trans retinoic acid as treatment in a case of primary myelofibrosis transforming into acute myeloid leukaemia

et al. 2019

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“…Myeloproliferative neoplasms (MPN) have been previously called myeloproliferative disorders are chronic, clonal hematopoietic stem cell group of disorders characterized by enhanced proliferation of one or more myeloid lineage cells (Sag et al, 2015;Yang et al, 2015). 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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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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“…Myeloproliferative neoplasms (MPN) are a group of diseases which are characterized by excessive production of one or more myeloid lineage cells arising from genetic mutations in multipotential hematopoietic stem cells in bone marrow (Sag et al, 2015;Yang et al, 2015). Idiopathic Primary myelofibrosis (PMF) is a heterogenous Philadelphia chromosome-negative MPN exemplified by cytopenias, progressive marrow fibrosis; extramedullary hematopoiesis associated with excessive production of inflammatory cytokines and shortened survival.…”

Section: Introductionmentioning

confidence: 99%

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

Sultan

Irfan²

2016

Asian Pacific Journal of Cancer Prevention

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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±19.2x10

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Methylated Alteration of SHP1 Complements Mutation of JAK2 Tyrosine Kinase in Patients with Myeloproliferative Neoplasm

Cited by 10 publications

References 31 publications

Decitabine combined with all-trans retinoic acid as treatment in a case of primary myelofibrosis transforming into acute myeloid leukaemia

Decitabine combined with all-trans retinoic acid as treatment in a case of primary myelofibrosis transforming into acute myeloid leukaemia

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

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

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