Evidence that prefibrotic myelofibrosis is aligned along a clinical and biological continuum featuring primary myelofibrosis
| العنوان: | Evidence that prefibrotic myelofibrosis is aligned along a clinical and biological continuum featuring primary myelofibrosis |
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| المؤلفون: | Vittorio Rosti, Paolo Catarsi, Letizia Lupo, Valentina Poletto, Gianluca Viarengo, Laura Villani, Rita Campanelli, Margherita Massa, Adriana Carolei, Elisa Bonetti, Antonina M. Isgrò, Umberto Magrini, Giovanni Barosi |
| المصدر: | PLoS ONE PLoS ONE, Vol 7, Iss 4, p e35631 (2012) |
| سنة النشر: | 2012 |
| مصطلحات موضوعية: | Male, Pathology, Epidemiology, DNA Mutational Analysis, CD34, Kaplan-Meier Estimate, Cohort Studies, Hematologic Cancers and Related Disorders, Fibrosis, Longitudinal Studies, Child, Polycythemia Vera, White Cells, Aged, 80 and over, Thrombocytosis, Multidisciplinary, medicine.diagnostic_test, Hematology, Middle Aged, Prognosis, Thrombosis, medicine.anatomical_structure, Phenotype, Cohort, Medicine, Female, Research Article, Adult, Platelets, medicine.medical_specialty, Adolescent, Clinical Research Design, Science, Mutation, Missense, World Health Organization, Young Adult, White blood cell, Biopsy, medicine, Humans, Myelofibrosis, Biology, Aged, Myeloproliferative Disorders, Population Biology, business.industry, Janus Kinase 2, medicine.disease, Hematopoiesis, Logistic Models, Primary Myelofibrosis, Bone marrow, business |
| الوصف: | PurposeIn 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.Patients and methodsWe investigated whether pre-MF may be aligned along a clinical and biological continuum in 683 consecutive patients who received a WHO diagnosis of PMF.ResultsAs 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.ConclusionPre-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. |
| تدمد: | 1932-6203 |
| الوصول الحر: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::b8a2cdc3c2189620be25f63326f8f66fTest https://pubmed.ncbi.nlm.nih.gov/22536419Test |
| حقوق: | OPEN |
| رقم الانضمام: | edsair.doi.dedup.....b8a2cdc3c2189620be25f63326f8f66f |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 19326203 |
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