Prospective study to evaluate the relevance of CALR allele burden monitoring as a molecular marker of disease progression.
adults (age ≥18 years),
affiliated to the national social security system,
with CALR mutated myeloproliferative neoplasm diagnosed between 2006 - 2020,
for which at least one sample is available at the time of diagnosis or before cytoreductive treatment,
patient with another active hematological disease or cancer at the time of diagnosis,
person subject to legal protection scheme or incapable of giving consent.
Single group
CALR allele burden quantification
Biological/Vaccine* DNA extraction from blood sample for CALR mutation quantification (fragment analysis) * at diagnosis and follow-up (inclusion period: 3 years) * max 1 sample/year * secondary outcome: mutational landscape by Next Generation Sequencing (NGS) analysis at diagnosis
For each disease, Hazard Ratio of the different trajectories of CALR allele burden to explain the time to onset of disease progression by the clinicobiological score.
Clinicobiological score : For ET, disease progression if ≥ 1 of: * leukocytosis \> 12 G/L or myelemia \> 10% or erythroblasts \> 1%, * anemia or thrombocytopenia not related to drug toxicity, * development or worsening of splenomegaly, * development of thrombocytosis (platelets \> 450 G/L) on cytoreductive therapy, * poor disease control (at least one change in therapy for reasons other than adverse events), * hematologic transformation or death related to hematologic pathology. For MF, disease progression if ≥ 1 of: * anemia \< 100 g/L, neutropenia \< 1 G/L, thrombocytopenia \< 100 G/L and/or development of general signs not previously present or recurring after improvement, * increase in leukocytosis \> 25 G/L not previously present, * appearance or aggravation of transfusion dependence, * appearance (\> 5 cm) or aggravation (\> 50%) of splenomegaly, * leukemic transformation or death related to the hematologic pathology.
A multinomial logistic model will be performed to identify the characteristics associated with the different trajectories of CALR allele burden (pathology, treatment, additional mutations, type of CALR mutation).
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University Hospital, Angers
Lead sponsor
Ligue contre le cancer, France
Collaborator
