Impact of Bone Marrow Fibrosis Grade in Post-Polycythemia Vera and Post-Essential Thrombocythemia Myelofibrosis. a Study of the Mysec Group in Blood
2019
AOU Città della Salute di Torino
Tipo pubblicazione
Conference Abstract
Autori/Collaboratori (28)Vedi tutti...
Mora B
Hematology, Ospedale di Circolo e Fondazione Macchi, ASST Sette Laghi, University of Insubria, Varese, Italy
Rumi E
Department of Molecular Medicine, IRCCS Policlinico San Matteo, University of Pavia, Pavia, Italy
Guglielmelli P
CRIMM, Center Research and Innovation of Myeloproliferative Neoplasms, University of Florence, Florence, Italy
et alii...
Abstract
Background: Polycythemia vera (PV) and essential thrombocythemia (ET) are myeloproliferative neoplasms that can progress to post-PV (PPV) myelofibrosis (MF) and post-ET (PET) MF, also known as secondary myelofibrosis (SMF). The 2009 International Working Group for Myelofibrosis Research and Treatment group (IWG-MRT) diagnostic criteria for SMF require the presence of grade 2 or 3 bone marrow fibrosis (BMF) according to the European classification. The prognostic value of BMF in primary MF has been recently explored. Aim of this study is to describe the relevance of BMF grading in terms of presentation and outcome in SMF. Methods: The MYSEC (Myelofibrosis Secondary to PV and ET Collaboration) project is an international retrospective collaboration that collected 805 SMF cases. BMF grade at time of SMF evolution was defined as grade 2 (BMF2) in case of a diffuse increase in reticulin with extensive intersections, occasional bundles of collagen and/or osteosclerosis, or as grade 3 (BMF3) if presenting with coarse bundles of collagen, often associated with osteosclerosis. Chi-square or Fisher exact test for categorical variables, Wilcoxon rank-sum test for continuous ones and multiple logistic regression analysis were applied in order to explore pattern of association between BMF grading and patients' characteristics. A Poisson regression model was applied to estimate incidence rate of events, together with 95% Confidence Interval (CI). Survival was estimated using the Kaplan-Meier method. Differences between BMF groups were compared by a Log-rank test. The MYSEC study was approved by the Review Board of each Institution and conducted in accordance with the Declaration of Helsinki. Results: Detailed information about BMF grade was available in 675 patients: BMF2 was reported in 443 (65.6%) and BMF3 in 232 (34.4%). We did not find any correlation between BMF grade and advanced age, marrow blasts percentage, spleen and liver size, presence of constitutional symptom
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DOI : 10.1182/blood-2019-124854
Keywords
collagen; endogenous compound; reticulin; silver; adult; advanced cancer; advisory committee; anemia; blast cell crisis; bone marrow biopsy; cancer growth; cancer patient; cancer prognosis; cancer survival; chromosome aberration; conference abstract; cumulative incidence; disease free interval; female; Fisher exact test; follow up; funding; gender; genotype; hemoglobin blood level; high risk population; human; human cell; Kaplan Meier method; leukocyte count; liver size; log rank test; major clinical study; male; mortality; myelofibrosis; osteosclerosis; phenotype; platelet count; polycythemia vera; protein expression; rank sum test; retrospective study; spleen size; thrombocythemia; thrombocytopenia; thrombosis; travel; univariate analysis;