Secondary Cancers in Myeloproliferative Neoplasms (MPN-K Study)

Study Overview
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Brief Summary
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The incidence of secondary cancer (SC) in patients with myeloproliferative neoplasms (MPN) is high and comparable to that of thrombosis. However, the identification of patient subgroups that might be at increased susceptibility of developing SC has not been systematically addressed. This international case-control study (MPN-K) is aimed to elucidate the prognostic role of JAK2V617F mutation in predicting the occurrence of SC in patients with classical MPN, polycythemia vera (PV), essential thrombocythemia (ET) and myelofibrosis (MF)

Detailed Description
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For each recruited case with concomitant diagnosis of myeloproliferative neoplasms (MPN) and secondary cancer each participating center will provide up 3 control patients (1 control for each case could be accepted but the optimal number is 3). Controls are patients with myeloproliferative neoplasms and no history of cancer. Each control will be matched to the paired case for sex, age (+/- 3 years), date of MPN diagnosis (+/- 5 years), and MPN disease duration (+/- 3 years). Each control is censored at the date of the secondary cancer occurrence in his matched case (index date). Data will be collected retrospectively from pre-existing medical records and reported by each center on a web-based and validated eCRF developed to record y all study data. In order to maintain patient privacy, all data records will be treated anonymously and no personal data to identify patient will be recorded: patients will be identified in the study by an alphanumeric code.

Official Title
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Secondary Cancers in Myeloproliferative Neoplasms (MPN-K Study)

Conditions
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Polycythemia Vera, Essential Thrombocythemia, Myelofibrosis

Intervention / Treatment
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* Other: JAK2V617F mutation


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Diagnosis of Philadelphia-negative Myeloproliferative Neoplasms (MPN) according to PVSG, 2008 and 2016 WHO criteria, including: Polycythemia Vera (PV) Essential Thrombocythemia (ET) Myelofibrosis (MF), including both primary and secondary MF Diagnosis performed between 1st January 2000 to 31 December 2016 Diagnosis of secondary cancer(s) performed concurrently or subsequently the diagnosis of MPN Exclusion Criteria: - Diagnosis of cancer occurred before the diagnosis of MPN (PV, ET, MF)
Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Cases<br>Patients with diagnosis of Myeloproliferative Neoplasms (MPN) including Polycythemia Vera, Essential thrombocytopenia or Myelofibrosis, exposed or not exposed to JAK2V617F mutation, who experienced secondary cancer(s) diagnosed at presentation of MPN or during the course of the myeloproliferative disease. | Other: JAK2V617F mutation<br>* JAK2 V617F is attached to the cytosolic juxtamembrane region of dimeric cytokine receptors, such as EpoR or MPL (TpoR); The JAK2V617F mutation results from a guanine to thymine change at nucleotide 1849 of the cDNA, in exon 14 of the gene. This valine is located at one of the predicted interfaces between JH1 and JH2 domains,<br>|
| Controls<br>Patients with diagnosis of Myeloproliferative Neoplasms (MPN) including Polycythemia Vera, Essential thrombocytopenia or Myelofibrosis, exposed or not exposed to JAK2V617F mutation, without history of secondary cancer. | Other: JAK2V617F mutation<br>* JAK2 V617F is attached to the cytosolic juxtamembrane region of dimeric cytokine receptors, such as EpoR or MPL (TpoR); The JAK2V617F mutation results from a guanine to thymine change at nucleotide 1849 of the cDNA, in exon 14 of the gene. This valine is located at one of the predicted interfaces between JH1 and JH2 domains,<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Number of patients with secondary cancers after diagnosis of Polycythemia Vera (PV), Essential Trombocythemia (ET) and Myelofibrosis (MF) | The ratio of number of patients showing JAK2V617F mutation on the number of patients not exposed to this mutation will be calculated in the group of subjects experiencing second cancer after diagnosis of PV, ET and MF (defined as 'cases') and related (odds ratio) with the ratio of patients exposed on those not exposed to JAK2V617F mutation in the group of subjects with no experience of secondary cancers after diagnosis of PV, ET and MF (this group is defined as 'Control' group) | 10 year from diagnosis of PV, ET or MF | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Number of patients with secondary cancers after diagnosis of Polycythemia Vera (PV), Essential Trombocythemia (ET) and Myelofibrosis (MF) in subgroups of subjects exposed to potential risk factors at diagnosis | Characteristics at diagnosis of patients with PV, ET and MF (including other mutations) with occurrence of second cancers (SC) are decribed and compared with those not experiencing SC after diagnosis of myloproliferative neoplasm; the ratio of number of patients exposed to potential risk factors on the number of subjects not exposed will be calculated in the group cases (as defined for primary outcome measure) and related (odds ratio) with the ratio of patient exposed versus not exposed calculated in the subgroup of control. | 10 year from diagnosis of PV, ET or MF | 
| Number of patients with secondary cancers after diagnosis of Polycythemia Vera (PV), Essential Trombocythemia (ET) and Myelofibrosis (MF) in the subgroups exposed to treatment | Group of patients exposed and not exposed to different class of treatments for PV, ET and MF are compared and related in the group of cases and control | 10 year from diagnosis of PV, ET or MF | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Myeloproliferative neoplasms, Secondary cancer, JAK2V617F mutation