Chronic "Philadelphia-negative" myeloproliferative syndromes are chronic blood disorders. They include essential thrombocythemia, polycythemia vera, and myelofibrosis. Myelofibrosis may arise de novo ("primary myelofibrosis") or represent the evolution of essential thrombocythemia or polycythemia vera ("secondary myelofibrosis").
The myelofibrotic stage-characterized, as the name implies, by the presence of bone marrow fibrosis (deposition of scar-like tissue)-is generally associated with a more severe and symptomatic disease. To date, the only way to assess fibrotic progression in these disorders is bone marrow biopsy.
The aim of this project is to evaluate whether the identification, tracking, and quantification of cells expressing a specific receptor (CCR2), a selective biomarker of fibrosis, may allow early and non-invasive identification of the fibrotic stage of the disease through:
* laboratory analysis on a blood sample (using flow cytometry) * use in PET-CT (positron emission tomography combined with computed tomography) of a tracer specific for the CCR2 receptor, capable of selectively binding to CCR2-expressing cells (⁶⁸Ga-DOTA-ECL1i).
Diagnosis of ET/PV/prePMF/overtPMF according to the WHO 2016 criteria and of SMF according to the IWG-MRT criteria (with histopathological data).
Age >= 18 yrs
ECOG performance status <=3
Pregnancy/breastfeeding
Ongoing therapy with immunomodulatory drugs (JAK-inhibitors, interferon).
For PET imaging, patients should be off any cytoreductive treatment for at least 3 months.
Antiplatelet agents are allowed