診斷MF

骨髓纖維化的診斷

如有下列症狀,建議你約見醫生進行骨髓纖維化檢查:3

· 體重半年間無故持續下降

· 易飽、骨痛

· 缺乏活力、盜汗、皮膚痕癢

· 發燒

醫生會安排你作身體檢查,察看脾臟有否腫大或出現貧血的跡象,並詢問你的病史,包括任何近日曾出現的症狀,而相關的診斷測試包括:⁵

全血細胞計數
(Complete Blood Count, CBC)
白血球和血小板數量升高,紅血球數量低於正常
造血生長因子 尿酸、膽紅素和乳酸脫氫酶水平升高
基因突變分析 檢查與骨髓纖維化相關的基因突變,尤其JAK2 V617F
骨髓活檢 把取出的骨髓樣本放在顯微鏡下進行檢查
影像學檢查 進行超聲波檢查以察看脾臟有否腫大

骨髓纖維化病情如何隨時間而變化?

大部分骨髓纖維化患者的病情會隨時間而惡化:⁶

部分患者的病情惡化速度會較快,醫生會根據風險分類來診斷和作出評估,並預期疾病進展的速度,繼而選擇適當的治療方案。⁷

參考資料:

  1. Primary Myelofibrosis. National Organization for Rare Disorders. Available at: https://rarediseases.org/rare-diseases/primary-myelofibrosis. Accessed 25 May 2022.
  2. Myeloproliferative Neoplasms (MPN) Research Funded by LLS. Leukemia & Lymphoma Society. Available at: lls.org/research/myeloproliferative-neoplasms-mpn-research-funded-lls. Accessed 25 May 2022.
  3. Myelofibrosis ‒ Symptoms & causes. Mayo Clinic. Available at: https://www.mayoclinic.org/diseases-conditions/myelofibrosis/symptoms-causes/syc-20355057. Accessed 7 February 2022.
  4. Song MK, et al. Understanding Splenomegaly in Myelofibrosis: Association with Molecular Pathogenesis. Int J Mol Sci. 2018;19(3):898.
  5. Myelofibrosis. Cleveland Clinic. Available at: https://my.clevelandclinic.org/health/diseases/15672-myelofibrosis. Accessed 4 March 2022.
  6. Tefferi A, et al. One thousand patients with primary myelofibrosis: the mayo clinic experience. Mayo Clin Proc. 2012;87(1):25-33.
  7. Ganget N, et al. DIPSS plus: a refined Dynamic International Prognostic Scoring System for primary myelofibrosis that incorporates prognostic information from karyotype, platelet count, and transfusion status. J Clin Oncol. 2011;29(4):392-7.
  8. Stein BL et al. Polycythemia vera disease burden: contributing factors, impact on quality of life, and emerging treatment options. Ann hematol. 2014; 93: 1965-1976.
  9. Scherber RM et al. Quality of Life in MPN Comes of Age as a Therapeutic Target. Curr Hematol Malig Rep. 2014;9:324-330.
  10. Gnessnammer M et al. Current and future treatment options for polycythemia vera. Ann Hematol. 2015; 94: 901-910.
  11. Myeloproliferative Neoplasms Version 2.2021. NCCN Guidelines. Available at: https://www.nccn.org/professionals/physician_gls/pdf/mpn.pdf. Accessed 7 February 2022.
  12. Leukaemia Care. Myelofibrosis. A Guide for Patients. Version 4. Printed October 2022.
  13. Erythropoietin-Stimulating Agents. Cleveland Clinic. Available at: https://my.clevelandclinic.org/health/drugs/14573-erythropoietin-stimulating-agents. Accessed 20 Jun 2022.
  14. Healthy Lifestyle Habits For A Better Quality Of Life. Leukemia & Lymphoma Society. Available at: https://www.lls.org/sites/default/files/National/USA/Pdf/Publications/FF7_Healthy_Behaviors.pdf. Accessed 7 February 2022.
  15. Follow-up Care And Survivorship. Leukemia & Lymphoma Society. Available at: https://www.lls.org/treatment/follow-care-and-survivorship. Accessed 7 February 2022.
  16. Staying Healthy through Diet and Physical Activity. Children’s Oncology Group. Available at: http://www.survivorshipguidelines.org/pdf/healthlinks/English/diet_and_physical_activity_Eng.pdf. Accessed 7 February 2022.
  17. Nutrition recommendations for MPN patients. Scherber RS et al. MPN Quality of Life Study Group. Available at https://www.mpnresearchfoundation.org/wp-content/uploads/2021/04/Nutr-Recs-for-MPNs_FINAL_12-4-19.pdf. Accessed 20 June 2022.
  18. Coping With Myelofibrosis. Cancer Care. Available at: https://www.cancercare.org/publications/197-coping_with_myelofibrosis. Accessed 7 February 2022.
  19. What Does It Mean To Be ‘Immunocompromised’?. Yale Medicine. Available at: https://www.yalemedicine.org/news/what-does-immunocompromised-mean. Accessed 7 February 2022.
  20. Prevention & Control ‒ Immunocompromised Persons. Centers for Disease Control and Prevention. Available at: https://www.cdc.gov/parasites/crypto/gen_info/prevent_ic.html. Accessed 7 February 2022.
  21. Coping With Myeloproliferative Neoplasms. Cancer Care. Available at: https://media.cancercare.org/publications/original/282-cfs_coping_mpns.pdf. Accessed 7 February 2022.
  22. Emanuel RM, et al. Myeloproliferative neoplasm (MPN) symptom assessment form total symptom score: prospective international assessment of an abbreviated symptom burden scoring system among patients with MPNs. J Clin Oncol. 2012;20;30(33):4098-103.
  23. Emanuel RM, et al. Myeloproliferative neoplasm (MPN) symptom assessment form total symptom score: prospective international assessment of an abbreviated symptom burden scoring system among patients with MPNs. J Clin Oncol. 2012;20;30(33) (supple 1):4098-103.