Reduced CXCR4-expression on CD34-positive blood cells predicts outcomes of persons with primary myelofibrosis

The expression of the CXCR4 chemokine receptor on CD34-positive blood cells is reduced in persons with primary myelofibrosis (PMF). We analyzed the relevance of cytofluorimetric assessment of the percentage of CD34-positive blood cells that had a positive CXCR4 surface expression (CD34/CXCR4-se) in...

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Bibliographic Details
Published in:Leukemia 2021-02, Vol.35 (2), p.468-475
Main Authors: Barosi, Giovanni, Rosti, Vittorio, Catarsi, Paolo, Villani, Laura, Abbà, Carlotta, Carolei, Adriana, Magrini, Umberto, Gale, Robert Peter, Massa, Margherita, Campanelli, Rita
Format: Article
Language:English
Subjects:
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Aged
Anemia
Antigens, CD34 - metabolism
Biomarkers
Biomarkers - metabolism
Blood
Blood cells
Blood Cells - metabolism
Cancer Research
Care and treatment
Case-Control Studies
CD34 antigen
Cell death
Chemokines
Cohort Studies
Critical Care Medicine
CXCR4 protein
Development and progression
Diagnosis
Diagnostic systems
Female
Fibrosis
Gene expression
Genetic aspects
Health aspects
Hematology
Hemoglobin
Humans
Intensive
Internal Medicine
Leukopenia
Male
Medicine
Medicine & Public Health
Middle Aged
Mutation
Myelofibrosis
Neoplasms
Oncology
Polycythemia
Polycythemia vera
Primary Myelofibrosis - diagnosis
Primary Myelofibrosis - metabolism
Prognosis
Receptors, CXCR4 - metabolism
Signal Transduction
Splenomegaly
Survival Rate
Thrombocytopenia
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Summary:The expression of the CXCR4 chemokine receptor on CD34-positive blood cells is reduced in persons with primary myelofibrosis (PMF). We analyzed the relevance of cytofluorimetric assessment of the percentage of CD34-positive blood cells that had a positive CXCR4 surface expression (CD34/CXCR4-se) in a large cohort of subjects with myeloproliferative neoplasms. Mean CD34/CXCR4-se was lower in subjects with PMF compared with those with essential thrombocythemia (ET) or polycythemia vera (PV). A cutoff value of 39% was associated with a diagnosis of pre-fibrotic PMF vs. ET with a positive predictive value of 97%. In PMF male sex, older age, and MPL mutation were independent correlates of reduced CD34/CXCR4-se and associated with a briefer interval to development of severe anemia, large splenomegaly, thrombocytopenia, leukopenia, elevated CD34-positive blood cells, blast transformation and death. We constructed a prognostic model including age >65 years, hemoglobin  50 × 10 6 /L, and CD34/CXCR4-se
ISSN:0887-6924
1476-5551
DOI:10.1038/s41375-020-0926-6