Essential Thrombocythemia, Myeloproliferative Disorder, Primary Myelofibrosis, Fibrotic Stage, Primary Myelofibrosis, Prefibrotic Stage
Conditions
Brief summary
Prospective study for the development of a non-invasive score for differentiating prefibrotic myelofibrosis from essential thrombocytosis and overt myelofibrosis.
Detailed description
The diagnostic criteria for myeloproliferative neoplasia have recently been revised in the WHO 2016 classification, which now recognizes a new entity between essential thrombocythemia (TE) and overt myelofibrosis (or primary myelofibrosis): prefibrotic myelofibrosis. Prefibrotic myelofibrosis patients seem to have a poorer prognosis compared to essential thrombocythemia in term of overall survival and myelofibrotic evolution. The dichotomy between these diseases is based on the bone marrow biopsy evaluation and is challenging with a lack of reproducibility showed in the literature. This study aims to develop a diagnostic non-invasive score for differentiating prefibrotic myelofibrosis from essential thrombocytosis and overt myelofibrosis. The parameters studied are : * spleen fibrosis by elastography * plasma cytokines levels * mutationnal landscape by NGS * CD34 circulating cells Bone marrow biopsy were reviewed by 2 anatomopathologists. Number of patients to be enrolled : 130
Interventions
DIAGNOSTIC_TESTNon-invasive diagnosis score
* spleen fibrosis by elastography * plasma cytokines levels * mutationnal landscape by NGS * CD34 circulating cells
Sponsors
University Hospital, Angers
Study design
Allocation
NA
Intervention model
SINGLE_GROUP
Primary purpose
DIAGNOSTIC
Masking
NONE
Eligibility
Sex/Gender
ALL
Age
18 Years to 99 Years
Healthy volunteers
No
Inclusion criteria
* Adult (18 years of age or older) with the diagnosis of essential thrombocytosis, prefibrotic myelofibrosis or overt myelofibrosis according to WHO 2016 criteria; * Patient who has not received specific treatment for neoplasms; * Patient who has signed the consent to participate in the study; * Patient who has consented to be included in the Malignant Haemopathies collection at the University Hospital of Angers or Brest and for whom the samples necessary for the study are available in the biocollection.
Exclusion criteria
* Patient with another hematologic neoplasm or progressive cancer at the time of diagnosis; * Patient with a diagnosis of secondary myelofibrosis; * Pregnant or breastfeeding woman; * Person prived of liberty by judicial or administrative decision; * Person subject to a legal protection measure;
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| WHO 2016 criteria for prefibrotic myelofibrosis, essential thrombocytosis and overt myelofibrosis diagnosis | At the time of diagnosis : disease classification | Assessment of the non-invasive diagnostic score against the WHO diagnosis |
Countries
France
Outcome results
None listed