骨髓纖維化

甚麼是骨髓纖維化 ?

骨髓纖維化 (Myelofibrosis, MF) 是骨髓增生性腫瘤 (Myeloproliferative neoplasm, MPN) 的其中一種。MPN與其他癌症一樣,都是由細胞不正常增生所致。MPN的病理特徵是骨髓製造過多的紅血球、白血球或血小板等細胞,並可分為以下三大類:1

相對於原發性骨髓纖維化,原發性血小板增多症和真性紅血球增多症的傷害性較低,但他們亦可以進展成繼發性骨髓纖維化。1,2  骨髓纖維化是由於骨髓(骨骼內的脂肪及軟組織)內的變異幹細胞過份增生,導致骨髓受損的疾病。當骨髓被傷疤組織取代時,正常的造血功能便會受到影響,引致一些症狀例如貧血及疲倦等。3

此外,骨髓纖維化會促使造血功能轉移至脾臟(脾臟位於肋骨左下方,負責過濾血液),令脾臟變大,引致腹部不適,使人容易有飽腹、腹脹或虛弱的感覺。4

骨髓纖維化有甚麼症狀?

患者可能會出現以下症狀。如出現腹痛,建議儘快求醫作檢查。

疲勞 廣泛性骨頭疼痛
夜間盜汗 活動力欠佳
容易有飽腹感 體溫超過37.8℃
搔癢 注意力下降
腹部不適 過去6個月非刻意的體重減輕

脾臟變大 4

在正常情況下,人體內大部分新的血液細胞都是由骨髓製造,但骨髓纖維化患者的骨髓無法正常運作,因此脾臟也要負擔起製造新血液細胞的工作並儲存這些細胞。故此,許多患者的脾臟會變大,這也是骨髓纖維化的主要病徵之一。

患者的脾臟腫大程度因人而異,但任何程度的腫大都不容忽視。如脾臟持續變大或會阻礙身體其他器官運作。

脾臟腫大可能引發的一些症狀:

• 腹部不適

• 很快出現飽腹感

• 左側肋骨下方疼痛

哪些人士患上骨髓纖維化的風險較高?

除了小部分病例是由真性紅血球增多症或原發性血小板增多症發展而成外,大部分骨髓纖維化都是原發性的。至於確切成因至今尚未清楚,但醫學界已知的高風險因素,包括:²

遺傳因素 ²
約一半的骨髓纖維化患者出現JAK2 V617F 基因變異
年齡 ²
骨髓纖維化患多為60-70歲人士
電離輻射 ³
經常或長期接觸高能量輻射,如醫療用X光。
化學物品 ³
例如苯及甲苯

如欲了解更多有關骨髓纖維化的詳情,請向你的醫生查詢,以獲取相關的支援群組、論壇或網頁等資訊。

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.