Evidence that prefibrotic myelofibrosis is aligned along a clinical and biological continuum featuring primary myelofibrosis.
<h4>Purpose</h4>In the WHO diagnostic classification, prefibrotic myelofibrosis (pre-MF) is included in the category of primary myelofibrosis (PMF). However, strong evidence for this position is lacking.<h4>Patients and methods</h4>We investigated whether pre-MF may be aligne...
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2012
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oai:doaj.org-article:2afb4bf2b4d34b6d8cf4b08c1c1f75892021-11-18T07:21:24ZEvidence that prefibrotic myelofibrosis is aligned along a clinical and biological continuum featuring primary myelofibrosis.1932-620310.1371/journal.pone.0035631https://doaj.org/article/2afb4bf2b4d34b6d8cf4b08c1c1f75892012-01-01T00:00:00Zhttps://www.ncbi.nlm.nih.gov/pmc/articles/pmid/22536419/?tool=EBIhttps://doaj.org/toc/1932-6203<h4>Purpose</h4>In the WHO diagnostic classification, prefibrotic myelofibrosis (pre-MF) is included in the category of primary myelofibrosis (PMF). However, strong evidence for this position is lacking.<h4>Patients and methods</h4>We investigated whether pre-MF may be aligned along a clinical and biological continuum in 683 consecutive patients who received a WHO diagnosis of PMF.<h4>Results</h4>As compared with PMF-fibrotic type, pre-MF (132 cases) showed female dominance, younger age, higher hemoglobin, higher platelet count, lower white blood cell count, smaller spleen index and higher incidence of splanchnic vein thrombosis. Female to male ratio and hemoglobin steadily decreased, while age increased from pre-MF to PMF- fibrotic type with early and to advanced bone marrow (BM) fibrosis. Likely, circulating CD34+ cells, LDH levels, and frequency of chromosomal abnormalities increased, while CXCR4 expression on CD34+ cells and serum cholesterol decreased along the continuum of BM fibrosis. Median survival of the entire cohort of PMF cases was 21 years. Ninety-eight, eighty-one and fifty-six percent of patients with pre-MF, PMF-fibrotic type with early and with advanced BM fibrosis, respectively, were alive at 10 years from diagnosis.<h4>Conclusion</h4>Pre-MF is a presentation mode of PMF with a very indolent phenotype. The major consequences of this contention is a new clinical vision of PMF, and the need to improve prognosis prediction of the disease.Giovanni BarosiVittorio RostiElisa BonettiRita CampanelliAdriana CaroleiPaolo CatarsiAntonina M IsgròLetizia LupoMargherita MassaValentina PolettoGianluca ViarengoLaura VillaniUmberto MagriniPublic Library of Science (PLoS)articleMedicineRScienceQENPLoS ONE, Vol 7, Iss 4, p e35631 (2012) |
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Medicine R Science Q |
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Medicine R Science Q Giovanni Barosi Vittorio Rosti Elisa Bonetti Rita Campanelli Adriana Carolei Paolo Catarsi Antonina M Isgrò Letizia Lupo Margherita Massa Valentina Poletto Gianluca Viarengo Laura Villani Umberto Magrini Evidence that prefibrotic myelofibrosis is aligned along a clinical and biological continuum featuring primary myelofibrosis. |
| description |
<h4>Purpose</h4>In the WHO diagnostic classification, prefibrotic myelofibrosis (pre-MF) is included in the category of primary myelofibrosis (PMF). However, strong evidence for this position is lacking.<h4>Patients and methods</h4>We investigated whether pre-MF may be aligned along a clinical and biological continuum in 683 consecutive patients who received a WHO diagnosis of PMF.<h4>Results</h4>As compared with PMF-fibrotic type, pre-MF (132 cases) showed female dominance, younger age, higher hemoglobin, higher platelet count, lower white blood cell count, smaller spleen index and higher incidence of splanchnic vein thrombosis. Female to male ratio and hemoglobin steadily decreased, while age increased from pre-MF to PMF- fibrotic type with early and to advanced bone marrow (BM) fibrosis. Likely, circulating CD34+ cells, LDH levels, and frequency of chromosomal abnormalities increased, while CXCR4 expression on CD34+ cells and serum cholesterol decreased along the continuum of BM fibrosis. Median survival of the entire cohort of PMF cases was 21 years. Ninety-eight, eighty-one and fifty-six percent of patients with pre-MF, PMF-fibrotic type with early and with advanced BM fibrosis, respectively, were alive at 10 years from diagnosis.<h4>Conclusion</h4>Pre-MF is a presentation mode of PMF with a very indolent phenotype. The major consequences of this contention is a new clinical vision of PMF, and the need to improve prognosis prediction of the disease. |
| format |
article |
| author |
Giovanni Barosi Vittorio Rosti Elisa Bonetti Rita Campanelli Adriana Carolei Paolo Catarsi Antonina M Isgrò Letizia Lupo Margherita Massa Valentina Poletto Gianluca Viarengo Laura Villani Umberto Magrini |
| author_facet |
Giovanni Barosi Vittorio Rosti Elisa Bonetti Rita Campanelli Adriana Carolei Paolo Catarsi Antonina M Isgrò Letizia Lupo Margherita Massa Valentina Poletto Gianluca Viarengo Laura Villani Umberto Magrini |
| author_sort |
Giovanni Barosi |
| title |
Evidence that prefibrotic myelofibrosis is aligned along a clinical and biological continuum featuring primary myelofibrosis. |
| title_short |
Evidence that prefibrotic myelofibrosis is aligned along a clinical and biological continuum featuring primary myelofibrosis. |
| title_full |
Evidence that prefibrotic myelofibrosis is aligned along a clinical and biological continuum featuring primary myelofibrosis. |
| title_fullStr |
Evidence that prefibrotic myelofibrosis is aligned along a clinical and biological continuum featuring primary myelofibrosis. |
| title_full_unstemmed |
Evidence that prefibrotic myelofibrosis is aligned along a clinical and biological continuum featuring primary myelofibrosis. |
| title_sort |
evidence that prefibrotic myelofibrosis is aligned along a clinical and biological continuum featuring primary myelofibrosis. |
| publisher |
Public Library of Science (PLoS) |
| publishDate |
2012 |
| url |
https://doaj.org/article/2afb4bf2b4d34b6d8cf4b08c1c1f7589 |
| work_keys_str_mv |
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