Myelofibrosis history and symptoms

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]

Overview

A significant proportion of patients with myelofibrosis can be asymptomatic. The hallmark of the disease is pancytopenia. A positive history of fatigue, recurring infections, and bleeding complications is suggestive of myelofibrosis. The most common symptom is fatigue which is prominent enough as it remarkably affects the quality of life. Fatigue, a result of anemia, leads to the associated complaints of weakness, palpitations, and dyspnea on exertion. Other nonspecific symptoms such as fever, night sweats, and weight loss can also be present at diagnosis.

History and Symptoms

History

Patients with myelofibrosis may have a positive history of:

  • Fatigue (the most commonly reported)[1][2][3]
  • Night sweats[4]
  • Bone pain[5]
  • Fever[6][7]
  • Weight loss[4]
  • Generalized abdominal pain[8][9]
  • Left subcostal pain
  • Early satiety

Common Symptoms

Common symptoms of myelofibrosis include:

Less Common Symptoms

References

  1. ↑ 1.0 1.1 Birgegard G, Samuelsson J, Ahlstrand E, Ejerblad E, Enevold C, Ghanima W, Hasselbalch H, Nielsen CH, Knutsen H, Pedersen OB, Sørensen A, Andreasson B (November 2018). "Inflammatory functional iron deficiency common in myelofibrosis, contributes to anaemia and impairs quality of life. From the Nordic MPN study Group". Eur. J. Haematol. doi:10.1111/ejh.13198. PMID 30472746.
  2. ↑ 2.0 2.1 Chahdi H, Oukabli M (2018). "[A special form of pancytopenia]". Pan Afr Med J (in French). 29: 209. doi:10.11604/pamj.2018.29.209.14055. PMC 6080970. PMID 30100963.
  3. ↑ 3.0 3.1 Tefferi A (December 2018). "Primary myelofibrosis: 2019 update on diagnosis, risk-stratification and management". Am. J. Hematol. 93 (12): 1551–1560. doi:10.1002/ajh.25230. PMID 30039550.
  4. ↑ 4.0 4.1 4.2 Tefferi, Ayalew (2000). "Myelofibrosis with Myeloid Metaplasia". New England Journal of Medicine. 342 (17): 1255–1265. doi:10.1056/NEJM200004273421706. ISSN 0028-4793.
  5. ↑ Gwaltney C, Paty J, Kwitkowski VE, Mesa RA, Dueck AC, Papadopoulos EJ, Wang L, Feliciano J, Coons SJ (August 2017). "Development of a harmonized patient-reported outcome questionnaire to assess myelofibrosis symptoms in clinical trials". Leuk. Res. 59: 26–31. doi:10.1016/j.leukres.2017.05.012. PMID 28544906.
  6. ↑ 6.0 6.1 Mitra D, Kaye JA, Piecoro LT, Brown J, Reith K, Mughal TI, Sarlis NJ (December 2013). "Symptom burden and splenomegaly in patients with myelofibrosis in the United States: a retrospective medical record review". Cancer Med. 2 (6): 889–98. doi:10.1002/cam4.136. PMC 3892393. PMID 24403262.
  7. ↑ 7.0 7.1 Mesa RA, Shields A, Hare T, Erickson-Viitanen S, Sun W, Sarlis NJ, Sandor V, Levy RS, Verstovsek S (August 2013). "Progressive burden of myelofibrosis in untreated patients: assessment of patient-reported outcomes in patients randomized to placebo in the COMFORT-I study". Leuk. Res. 37 (8): 911–6. doi:10.1016/j.leukres.2013.04.017. PMID 23684482.
  8. ↑ 8.0 8.1 Pizzi M, Gergis U, Chaviano F, Orazi A (September 2016). "The effects of hematopoietic stem cell transplant on splenic extramedullary hematopoiesis in patients with myeloproliferative neoplasm-associated myelofibrosis". Hematol Oncol Stem Cell Ther. 9 (3): 96–104. doi:10.1016/j.hemonc.2016.07.002. PMID 27521149.
  9. ↑ 9.0 9.1 Mohyuddin GR, Yacoub A (2016). "Primary Myelofibrosis Presenting as Extramedullary Hematopoiesis in a Transplanted Liver Graft: Case Report and Review of the Literature". Case Rep Hematol. 2016: 9515404. doi:10.1155/2016/9515404. PMC 4739215. PMID 26885416.
  10. ↑ Xu J, Xu Z, Wang J, Li B, Sun X, Qin T, Zhang Y, Zhang H, Fang L, Pan L, Hu N, Qu S, Xiao Z (January 2016). "[The assessment of symptomatic burden among Ph/BCR- ABL negative myeloproliferative neoplasm patients]". Zhonghua Xue Ye Xue Za Zhi (in Chinese). 37 (1): 26–9. doi:10.3760/cma.j.issn.0253-2727.2016.01.005. PMID 26876249.
  11. ↑ 11.0 11.1 Magyari F, Bedekovics J, Décsy J, Ilonczai P, Illés Á, Simon Z (April 2018). "[Investigation and treatment of prefibrotic/early primary myelofibrosis. A case study]". Orv Hetil (in Hungarian). 159 (15): 603–609. doi:10.1556/650.2018.30995. PMID 29631428.
  12. ↑ Sultan S, Irfan SM (2015). "Primary Idiopathic Myelofibrosis: Clinico-Epidemiological Profile and Risk Stratification in Pakistani Patients". Asian Pac. J. Cancer Prev. 16 (18): 8629–31. PMID 26745128.
  13. ↑ Johansson P, Mesa R, Scherber R, Abelsson J, Samuelsson J, Birgegård G, Andréasson B (March 2012). "Association between quality of life and clinical parameters in patients with myeloproliferative neoplasms". Leuk. Lymphoma. 53 (3): 441–4. doi:10.3109/10428194.2011.619608. PMID 21883029.
  14. ↑ Harrison CN, Mesa RA, Kiladjian JJ, Al-Ali HK, Gisslinger H, Knoops L, Squier M, Sirulnik A, Mendelson E, Zhou X, Copley-Merriman C, Hunter DS, Levy RS, Cervantes F, Passamonti F, Barbui T, Barosi G, Vannucchi AM (July 2013). "Health-related quality of life and symptoms in patients with myelofibrosis treated with ruxolitinib versus best available therapy". Br. J. Haematol. 162 (2): 229–39. doi:10.1111/bjh.12375. PMID 23672349.
  15. ↑ Hoffman, Ronald (2018). Hematology : basic principles and practice. Philadelphia, PA: Elsevier. ISBN 9780323357623.
  16. ↑ Finazzi G, Vannucchi AM, Barbui T (November 2018). "Prefibrotic myelofibrosis: treatment algorithm 2018". Blood Cancer J. 8 (11): 104. doi:10.1038/s41408-018-0142-z. PMC 6221891. PMID 30405096.
  17. ↑ Hofmann I, Geer MJ, Vögtle T, Crispin A, Campagna DR, Barr A, Calicchio ML, Heising S, van Geffen JP, Kuijpers M, Heemskerk J, Eble JA, Schmitz-Abe K, Obeng EA, Douglas M, Freson K, Pondarré C, Favier R, Jarvis GE, Markianos K, Turro E, Ouwehand WH, Mazharian A, Fleming MD, Senis YA (September 2018). "Congenital macrothrombocytopenia with focal myelofibrosis due to mutations in human G6b-B is rescued in humanized mice". Blood. 132 (13): 1399–1412. doi:10.1182/blood-2017-08-802769. PMID 29898956. Vancouver style error: initials (help)
  18. ↑ Kc D, Falchi L, Verstovsek S (October 2017). "The underappreciated risk of thrombosis and bleeding in patients with myelofibrosis: a review". Ann. Hematol. 96 (10): 1595–1604. doi:10.1007/s00277-017-3099-2. PMC 5693670. PMID 28808761.
  19. ↑ Complications of primary myelofibrosis. Dr Henry Knipe and Dr Yuranga Weerakkody et al. Radiopaedia 2016. http://radiopaedia.org/articles/myelofibrosis. Accessed on March 10, 2016
  20. ↑ Yü TF (October 1965). "Secondary gout associated with myeloproliferative diseases". Arthritis Rheum. 8 (5): 765–71. PMID 5216775.
  21. ↑ Nagai Y, Ikebe K, Ito K, Nishizawa T, Akaoka I, Muranaka M, Horiuchi Y (March 1978). "[A case of secondary gout associated with myelofibrosis following polycythemia vera (author's transl)]". Rinsho Ketsueki (in Japanese). 19 (3): 226–33. PMID 702809.
  22. ↑ Yu T, Weinreb N, Wittman R, Wasserman LR (February 1976). "Secondary gout associated with chronic myeloproliferative disorders". Semin. Arthritis Rheum. 5 (3): 247–56. PMID 1062009.
  23. ↑ Hoffman, Ronald (2018). Hematology : basic principles and practice. Philadelphia, PA: Elsevier. ISBN 9780323357623.
  24. ↑ Henry M, Chitlur M, Rajpurkar M, Mastropietro CW, Poulik J, Ravindranath Y (May 2014). "Myelofibrosis, hepatic extramedullary hematopoiesis and ascites associated with vitamin D deficiency in early infancy". J. Pediatr. Hematol. Oncol. 36 (4): 319–21. doi:10.1097/MPH.0b013e31828e548a. PMID 23619118.
  25. ↑ 25.0 25.1 25.2 25.3 Symptoms of myelofibrosis. US National Library of Medicine 2016. https://www.nlm.nih.gov/medlineplus/ency/article/000531.htm. Accessed on March 7, 2016
  26. ↑ Tokai K, Miyatani H, Yoshida Y, Yamada S (July 2012). "Multiple esophageal variceal ruptures with massive ascites due to myelofibrosis-induced portal hypertension". World J. Gastroenterol. 18 (28): 3770–4. doi:10.3748/wjg.v18.i28.3770. PMC 3406433. PMID 22851873.
  27. ↑ Bĕlohlávek J, Schwarz J, Jirásek A, Krajina A, Polák F, Hrubý M (March 2001). "Idiopathic myelofibrosis complicated by portal hypertension treated with a transjugular intrahepatic portosystemic shunt (TIPS)". Wien. Klin. Wochenschr. 113 (5–6): 208–11. PMID 11293952.
  28. ↑ Mughal TI, Vaddi K, Sarlis NJ, Verstovsek S (2014). "Myelofibrosis-associated complications: pathogenesis, clinical manifestations, and effects on outcomes". Int J Gen Med. 7: 89–101. doi:10.2147/IJGM.S51800. PMC 3912063. PMID 24501543.
  29. ↑ Doki N, Irisawa H, Takada S, Sakura T, Miyawaki S (2007). "Transjugular intrahepatic portosystemic shunt for the treatment of portal hypertension due to idiopathic myelofibrosis". Intern. Med. 46 (4): 187–90. PMID 17301514.
  30. ↑ Hoffman, Ronald (2018). Hematology : basic principles and practice. Philadelphia, PA: Elsevier. ISBN 9780323357623.
  31. ↑ Fareed S, Nashwan AJ, Abu Jarir S, Husain A, Suliman DS, Ibrahim F, Moustafa A, Akhter MS, Yassin MA (August 2017). "Spinal Abscess Caused by Salmonella Bacteremia in a Patient with Primary Myelofibrosis". Am J Case Rep. 18: 859–864. PMC 5551928. PMID 28775247.
  32. ↑ Karigane D, Kikuchi T, Sakurai M, Kato J, Yamane Y, Hashida R, Abe R, Hatano M, Hasegawa N, Wakayama M, Shibuya K, Okamoto S, Mori T (July 2018). "Invasive hepatic mucormycosis: A case report and review of the literature". J. Infect. Chemother. doi:10.1016/j.jiac.2018.06.013. PMID 30057341.
  33. ↑ Miyata T, Masuzawa M, Katsuoka K, Higashihara M (July 2008). "Cutaneous extramedullary hematopoiesis in a patient with idiopathic myelofibrosis". J. Dermatol. 35 (7): 456–61. doi:10.1111/j.1346-8138.2008.00502.x. PMID 18705835.
  34. ↑ Corella F, Barnadas MA, Bordes R, Curell R, Espinosa I, Vergara C, Alomar A (May 2008). "[A case of cutaneous extramedullary hematopoiesis associated with idiopathic myelofibrosis]". Actas Dermosifiliogr (in Spanish; Castilian). 99 (4): 297–300. PMID 18394406.
  35. ↑ Kwon KS, Lee JB, Jang HS, Chung TA, Oh CK (June 1999). "A case of cutaneous extramedullary hematopoiesis in myelofibrosis with a preponderance of eosinophilic precursor cells". J. Dermatol. 26 (6): 379–84. PMID 10405485.
  36. ↑ Ruberto E, Espinola R, Brusco J, Bacchiocchi M, Zoppi J, Paz R (April 1995). "[Idiopathic myelofibrosis with extramedullary hematopoiesis foci in the skin and testicles. Report of a case]". Sangre (Barc) (in Spanish; Castilian). 40 (2): 157–60. PMID 7784946.
  37. ↑ Panda A, Chandrashekhara SH, Nambirajan A, Mishra P (December 2016). "Idiopathic myelofibrosis with disseminated hepatosplenic, mesenteric, renal and pulmonary extramedullary haematopoeisis, portal hypertension and tuberculosis: initial presentation and 2 years follow-up". BMJ Case Rep. 2016. doi:10.1136/bcr-2016-217854. PMID 28011890.
  38. ↑ Sakatoku K, Takeoka Y, Araki T, Miura A, Fujitani Y, Yamamura R, Miyagi Y, Senzaki H, Ohta K (2017). "Lymphocyte-depleted classical Hodgkin lymphoma accompanied by myelofibrosis". Rinsho Ketsueki (in Japanese). 58 (7): 772–775. doi:10.11406/rinketsu.58.772. PMID 28781273.
  39. ↑ Aljabry MS, Asiri S, Elsafi T, Elyamany G (May 2018). "Generalized lymphadenopathy secondary to isolated extramedullary hematopoiesis as an initial manifestation of primary myelofibrosis". Hematol Rep. 10 (2): 7588. doi:10.4081/hr.2018.7588. PMC 6036984. PMID 30046414.
  40. ↑ Eckert R, Huberty J, Gowin K, Mesa R, Marks L (December 2017). "Physical Activity as a Nonpharmacological Symptom Management Approach in Myeloproliferative Neoplasms: Recommendations for Future Research". Integr Cancer Ther. 16 (4): 439–450. doi:10.1177/1534735416661417. PMC 5739136. PMID 27458250.


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