Initial bone marrow reticulin fibrosis in polycythemia vera exerts an impact on clinical outcome

• Published in: Blood Vol. 119; no. 10; pp. 2239 - 2241
• Main Authors: Barbui, Tiziano, Thiele, Jürgen, Passamonti, Francesco, Rumi, Elisa, Boveri, Emanuela, Randi, Maria Luigia, Bertozzi, Irene, Marino, Filippo, Vannucchi, Alessandro M., Pieri, Lisa, Rotunno, Giada, Gisslinger, Heinz, Gisslinger, Bettina, Müllauer, Leonhard, Finazzi, Guido, Carobbio, Alessandra, Gianatti, Andrea, Ruggeri, Marco, Nichele, Ilaria, D'Amore, Emanuele, Rambaldi, Alessandro, Tefferi, Ayalew
• Format: Journal Article
• Language: English
• Published: Washington, DC Elsevier Inc 08-03-2012; Americain Society of Hematology
• Subjects: Biological and medical sciences; Bone Marrow - metabolism; Bone Marrow - pathology; Disease Progression; Diseases of red blood cells; Fibrosis; Follow-Up Studies; Hematologic and hematopoietic diseases; Humans; Janus Kinase 2 - genetics; Leukemia - complications; Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis; Medical sciences; Mutation; Polycythemia Vera - complications; Polycythemia Vera - genetics; Polycythemia Vera - pathology; Polycythemias; Primary Myelofibrosis - complications; Prognosis; Reticulin - metabolism; Survival Analysis; Thrombosis - complications; Myeloproliferative syndrome; Prognosis; Hematology; Fibrosis; Bone marrow; Polycythemia vera; Hemopathy
• ISSN: 0006-4971; 1528-0020
• DOI: 10.1182/blood-2011-11-393819

We examined the prevalence and prognostic relevance of bone marrow reticulin fibrosis in 526 patients with World Health Organization–defined polycythemia vera evaluated at the time of initial diagnosis. Seventy-four patients (14%) displayed mostly grade 1 reticulin fibrosis, with only 2 cases showing higher-grade fibrosis. Presenting clinical and laboratory characteristics, including JAK2V617F allele burden, between patients with and without fibrosis were similar for the most part, with the exception of a higher prevalence of palpable splenomegaly in patients with fibrosis (P < .01). Patients with fibrosis were less prone to experience thrombosis during their clinical course (1.1 vs 2.7 per 100 patient-years; P = .03) and more prone to develop post-polycythemia vera myelofibrosis (2.2 vs 0.8 per 100 patient-years; P = .01). There was no significant difference between the 2 groups in terms of overall or leukemia-free survival. The present study clarifies the incidence, degree, and prognostic relevance of bone marrow fibrosis obtained at time of initial diagnosis of polycythemia vera.