Patients of Myelodysplastic Syndrome with Mild/Moderate Myelofibrosis and a Monosomal Karyotype are Independently Associated with an Adverse Prognosis: Long-Term Follow-Up Data

Abstract

Na Wang, 1– 3 Hongzhi Xu, 1, 2 Qing Li, 1, 2 Xiaosheng Fang, 1, 2 Jie Liu, 1, 2 Xiaohui Sui, 1, 2 Lingyan Zhang, 1, 2 Yujie Jiang, 1, 2 Xin Wang 1– 5 1Department of Hematology, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Jinan, Shandong 250021, People’s Republic of China; 2Department of Hematology, Shandong Provincial Hospital, Cheeloo College of Medicine, Shandong University, Jinan, Shandong 250021, People’s Republic of China; 3School of Medicine, Shandong University, Jinan, Shandong 250012, People’s Republic of China; 4Shandong Provincial Engineering Research Center of Lymphoma, Jinan, Shandong 250021, People’s Republic of China; 5National Clinical Research Center for Hematologic Diseases, Jinan, Shandong 250021, People’s Republic of ChinaCorrespondence: Xin WangDepartment of Hematology, Shandong Provincial Hospital, Cheeloo College of Medicine, Shandong University, No. 324 Jingwu Road, Jinan, Shandong 250021, People’s Republic of ChinaTel [phone]Fax [phone]Email [email protected]: The aim of our study was to evaluate the clinical characteristics of myelodysplastic syndrome (MDS) patients with concomitant mild-to-moderate myelofibrosis (MF), and to assess its independent prognostic role in MDS patients diagnosed by World Health Organization 2016 classification (WHO2016C) with long-term follow-up.Patients and Methods: A total of 157 patients with primary MDS associated with or without MF were examined retrospectively with long-term follow-up. MF graded as MF-1/MF-2 was defined as “mild/moderate”. Cytogenetics testing and fluorescence in situ hybridization (FISH) were also conducted in all MDS patients.Results: Thirty-four (21.7%) of 157 MDS patients had MF. Also, 24 (15.3%) MDS patients based on WHO2016 criteria were defined as MF-1 and 10 (6.4%) as MF-2. MDS patients with MF-1/2 had a higher prevalence of death (p=0.002), leukemic progression (p=0.013), O blood type (p=0.039) as well as less hypercellular proliferation (p< 0.001) and less supportive treatment (p=0.003) compared with those without mild/moderate MF. Cytogenetics testing did not show a significant difference between MDS patients with and without MF. Multivariate analyses showed that MF (mild/moderate), a monosomal karyotype (MK) and % bone-marrow blasts were independently associated with shorter overall survival (OS) and progression-free survival (PFS). Age was an independent indicator of the adverse OS of MDS patients. Compared with those without MF, MDS patients with mild/moderate MF were significantly associated with worse OS and PFS in MK-negative subgroups and relatively low-risk Revised International Prognostic Scoring System for Myelodysplastic Syndromes (IPSS-R) stratification in long-term follow-up.Conclusion: Mild/moderate myelofibrosis and monosomal karyotype are independent indicators of a poor clinical outcome in MDS patients. In long-term follow-up, MDS with mild/moderate MF can be a prognostic marker for MDS patients with a specific MK stratification and IPSS-R stratification.Keywords: myelodysplastic syndromes, myelofibrosis, prognosis, cytogenetics

Keywords

• myelodysplastic syndromes
• myelofibrosis
• prognosis
• cytogenetics