CLINICAL AND LABORATORY PROFILES OF PATIENTS WITH PRIMARY MYELOFIBROSIS AT THE NATIONAL INSTITUTE OF HEMATOLOGY AND BLOOD TRANSFUSION BETWEEN 2019 AND 2024
Main Article Content
Abstract
Objective: To describle the clinical and laboratory profiles of patients with primary myelofibrosis (PMF) at the National Institute of Hematology and Blood Transfusion (NIHBT) between 2019 and 2024.
Methods: A cross-sectional descriptive study was conducted on 181 PMF patients managed at the NIHBT from January 2019 to December 2024.
Results: The cohort’s mean age was 62.7 ± 11.8 years, with a predominance of patients aged over 60. Constitutional symptoms were prevalent, including fatigue (89%), early satiety (70.2%), abdominal discomfort (70.2%), left upper quadrant pain (70.2%), and weight loss (63%). Physical examination revealed a high incidence of splenomegaly (95.6%) and anemia (89%).
Laboratory findings indicated that 90.6% of patients presented with anemia (Hb≤120 g/L). Platelet counts were variable: 26.5% exhibited thrombocytosis (≥450 G/L), while 14.9% had thrombocytopenia (< 50 G/L). Leukocytosis (≥11 G/L) was observed in 51.4% of the cases. Bone marrow evaluation showed erythroid hypoplasia in 85.6% of patients; granulocytic lineage was increased in 42.5% and decreased in 17.1%, while 73.5% demonstrated megakaryocytic hyperplasia. Bone marrow fibrosis was classified as Grade II in 64.6% and Grade III in 35.4% of patients. The prevalence of the JAK2 mutation was 67.1%, whereas CALR and MPL mutations were identified in 3.9% and 1.1% of the cohort, respectively.
Conclusion: Primary myelofibrosis patients present with heterogeneous clinical manifestations, characterized by high frequencies of splenomegaly and anemia. Laboratory analyses frequently reveal trilineage hematopoietic dysregulation in the bone marrow.
Article Details
Keywords
Primary myelofibrosis, National Institute of Hematology and Blood Transfusion, Vietnam.
References
2. Arber DA, Orazi A, Hasserjian R, Thiele J, Borowitz MJ, Le Beau MM, et al. The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood. 2016;127(20):2391-405.
3. de Freitas RM, da Costa Maranduba CM. Myeloproliferative neoplasms and the JAK/STAT signaling pathway: an overview. Revista brasileira de hematologia e hemoterapia. 2015;37(5):348-53.
4. Tefferi A. Myelofibrosis with myeloid metaplasia. The New England journal of medicine. 2000;342(17):1255-65.
5. Tefferi A, Vainchenker W. Myeloproliferative neoplasms: molecular pathophysiology, essential clinical understanding, and treatment strategies. Journal of clinical oncology : official journal of the American Society of Clinical Oncology. 2011;29(5):573-82.
6. Phạm Thị Yến Thư. Một số đặc điểm lâm sàng và xét nghiệm máu ngoại vi ở bệnh nhân xơ tủy nguyên phát tại Viện Huyết học - Truyền máu Trung Ương. Tạp chí Y học Việt Nam. 2023;530(1):88-91.
7. Nguyễn Ngọc Dũng, Hải Quân. Một số đặc điểm của tủy xương ở bệnh nhân xơ tủy nguyên phát tại Viện Huyết học - Truyền máu Trung Ương. Tạp chí Y học Việt Nam. 2023;530(1):230-3.
8. Barbui T, Thiele J, Gisslinger H, Kvasnicka HM, Vannucchi AM, Guglielmelli P, et al. The 2016 WHO classification and diagnostic criteria for myeloproliferative neoplasms: document summary and in-depth discussion. Blood cancer journal. 2018;8(2):15.
9. Thiele J, Kvasnicka HM, Orazi A. Bone marrow histopathology in myeloproliferative disorders--current diagnostic approach. Seminars in hematology. 2005;42(4):184-95.
10. Verstovsek S, Yu J, Scherber RM, Pandya S, Dieyi C, Chen C-C, et al. Changes in the Incidence and Overall Survival of Patients with Myeloproliferative Neoplasms between 2002 and 2016 in the United States. Blood. 2020;136:12-3.
11. Nguyễn Vũ Bảo Anh. Nghiên cứu đặc điểm lâm sàng, xét nghiệm và điều trị một số bệnh tăng sinh tủy ác tính giai đoạn 2015-2018 tại Viện Huyết học - Truyền máu Trung Ương. Hà Nội: Đại học Y Hà Nội; 2023.
12. Cervantes F, Dupriez B, Pereira A, Passamonti F, Reilly JT, Morra E, et al. New prognostic scoring system for primary myelofibrosis based on a study of the International Working Group for Myelofibrosis Research and Treatment. Blood. 2009;113(13):2895-901.
13. Gong X, Lu X, Xiao X, Wang W, Yang J, Fu Y, et al. Clinicopathologic characteristics of prefibrotic-early primary myelofibrosis in Chinese patients. Hum Pathol. 2014;45(3):498-503.
14. Vũ Đức Bình, Lê Thị Thu. Bước đầu đánh giá hiệu quả điều trị bằng ruxolitinib ở bệnh nhân xơ tủy nguyên phát tại Viện Huyết học - Truyền máu Trung Ương giai đoạn 2019-2024. Tạp chí Y học Việt Nam. 2025;550(1):363-8.
15. Yap YY, Law KB, Sathar J, Lau NS, Goh AS, Chew TK, et al. The epidemiology and clinical characteristics of myeloproliferative neoplasms in Malaysia. Experimental hematology & oncology. 2018;7:31.
16. Song J, Hussaini M, Zhang H, Shao H, Qin D, Zhang X, et al. Comparison of the Mutational Profiles of Primary Myelofibrosis, Polycythemia Vera, and Essential Thrombocytosis. Am J Clin Pathol. 2017;147(5):444-52.