UNLABELLED: Marrow fibrosis (MF) has rarely been studied in myelodysplastic syndromes (MDS). There are no data on occurrence and significance of MF in the context of the World Health Organization (WHO) classification of disease. In total, 349 bone marrow biopsies from 200 patients with primary MDS were examined for MF and its prognostic relevance. MF correlated with multilineage dysplasia, more severe thrombopenia, higher probability of a clonal karyotype abnormality, and higher percentages of blasts in the peripheral blood (P<0.002). Its frequency varied markedly between different MDS types ranging from 0 (RARS) to 16% (RCMD, RAEB, P<0.007). Two patients with MF showed a Janus kinase-2 mutation (V617F). Patients with MF suffered from marrow failure significantly earlier with shortening of the survival time down to 0.5 (RAEB-1/-2), and 1-2 (RCMD, RA) years in median (P<0.00005). The prognostic relevance of MF was independent of the International Prognostic Scoring System and the classification of disease. CONCLUSION: The risk of MF Differs markedly between various subtypes of MDS. MF indicates an aggressive course with a significantly faster progression to fatal marrow failure and should therefore be considered in diagnosis, prognosis and treatment of disease.
UNLABELLED: Marrow fibrosis (MF) has rarely been studied in myelodysplastic syndromes (MDS). There are no data on occurrence and significance of MF in the context of the World Health Organization (WHO) classification of disease. In total, 349 bone marrow biopsies from 200 patients with primary MDS were examined for MF and its prognostic relevance. MF correlated with multilineage dysplasia, more severe thrombopenia, higher probability of a clonal karyotype abnormality, and higher percentages of blasts in the peripheral blood (P<0.002). Its frequency varied markedly between different MDS types ranging from 0 (RARS) to 16% (RCMD, RAEB, P<0.007). Two patients with MF showed a Janus kinase-2 mutation (V617F). Patients with MF suffered from marrow failure significantly earlier with shortening of the survival time down to 0.5 (RAEB-1/-2), and 1-2 (RCMD, RA) years in median (P<0.00005). The prognostic relevance of MF was independent of the International Prognostic Scoring System and the classification of disease. CONCLUSION: The risk of MF Differs markedly between various subtypes of MDS. MF indicates an aggressive course with a significantly faster progression to fatal marrow failure and should therefore be considered in diagnosis, prognosis and treatment of disease.
Authors: Nicolaus Kröger; Tatjana Zabelina; Anja van Biezen; Ronald Brand; Dietger Niederwieser; Rodrigo Martino; Zi Yi Lim; Francesco Onida; Christoph Schmid; Laurent Garderet; Marie Robin; Michael van Gelder; Reinhard Marks; Argiris Symeonidis; Guido Kobbe; Theo de Witte Journal: Haematologica Date: 2010-10-22 Impact factor: 9.941
Authors: Katherine R Calvo; Donald C Vinh; Irina Maric; Weixin Wang; Pierre Noel; Maryalice Stetler-Stevenson; Diane C Arthur; Mark Raffeld; Amalia Dutra; Evgenia Pak; Kyungjae Myung; Amy P Hsu; Dennis D Hickstein; Stefania Pittaluga; Steven M Holland Journal: Haematologica Date: 2011-04-20 Impact factor: 9.941
Authors: Bin Fu; Chi Young Ok; Maitrayee Goswami; Wei Xei; Jesse M Jaso; Tariq Muzzafar; Carlos Bueso-Ramos; Srdan Verstovsek; Guillermo Garcia-Manero; L Jeffrey Medeiros; Sa A Wang Journal: Ann Hematol Date: 2013-05-10 Impact factor: 3.673
Authors: Peter L Greenberg; Heinz Tuechler; Julie Schanz; Guillermo Sanz; Guillermo Garcia-Manero; Francesc Solé; John M Bennett; David Bowen; Pierre Fenaux; Francois Dreyfus; Hagop Kantarjian; Andrea Kuendgen; Alessandro Levis; Luca Malcovati; Mario Cazzola; Jaroslav Cermak; Christa Fonatsch; Michelle M Le Beau; Marilyn L Slovak; Otto Krieger; Michael Luebbert; Jaroslaw Maciejewski; Silvia M M Magalhaes; Yasushi Miyazaki; Michael Pfeilstöcker; Mikkael Sekeres; Wolfgang R Sperr; Reinhard Stauder; Sudhir Tauro; Peter Valent; Teresa Vallespi; Arjan A van de Loosdrecht; Ulrich Germing; Detlef Haase Journal: Blood Date: 2012-06-27 Impact factor: 22.113
Authors: Carolina Leite; Lucas Delmonico; Gilda Alves; Romario José Gomes; Mariana Rodrigues Martino; Aline Rodrigues da Silva; Aline Dos Santos Moreira; Maria Christina Maioli; Luciano Rios Scherrer; Elenice Ferreira Bastos; Roberto Irineu; Maria Helena Ornellas Journal: Biomed Rep Date: 2017-08-09
Authors: Sanam Loghavi; Alyaa Al-Ibraheemi; Zhuang Zuo; Guillermo Garcia-Manero; Mariko Yabe; Sa A Wang; Hagop M Kantarjian; Cameron C Yin; Roberto N Miranda; Raja Luthra; L Jeffrey Medeiros; Carlos E Bueso-Ramos; Joseph D Khoury Journal: Br J Haematol Date: 2015-06-30 Impact factor: 6.998
Authors: M G Della Porta; E Travaglino; E Boveri; M Ponzoni; L Malcovati; E Papaemmanuil; G M Rigolin; C Pascutto; G Croci; U Gianelli; R Milani; I Ambaglio; C Elena; M Ubezio; M C Da Via'; E Bono; D Pietra; F Quaglia; R Bastia; V Ferretti; A Cuneo; E Morra; P J Campbell; A Orazi; R Invernizzi; M Cazzola Journal: Leukemia Date: 2014-05-20 Impact factor: 11.528