Myeloproliferative neoplasm natural history, complications, and prognosis

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

Overview

If left untreated, patients with myeloproliferative neoplasm may progress to develop weight loss, fever, and night sweats. Common complications of myeloproliferative neoplasm include splenomegaly, bleeding, and thrombosis. Prognosis is generally good with treatment, and the 3-year survival rate of patients with myeloproliferative neoplasm is approximately 35%.[1][2]

Natural History

The symptoms of myeloproliferative neoplasm usually develop in the sixth decade of life and start with symptoms such as anorexia, weight loss, and fatigue. The onset of symptoms is usually insidious, and the symptoms do not frequently cause many problems initially. The disease progresses quite slowly, typically over a period of months to years. However, without treatment, patients will develop severe symptoms, such as abdominal pain, bruising, bleeding, fever, and infection which may eventually lead to death.[1][2] The natural history of myeloproliferative neoplasm is dependent upon the subtype of myeloproliferative neoplasm and by the prognostic group. The natural history of myelodysplastic syndrome / myeloproliferative neoplasm unclassifiable (MDS/MPN, U) is poor.[3] In general, the natural history of patients above the age of 60, the presence of a JAK2 mutation, and a history of thrombosis is worse than the natural history of patients under age 60, the absence of JAK2 mutation, and no history of thrombosis.[4]

Complications

Myeloproliferative neoplasm may lead to the following complications:[5][2][6]

  • Spleen

Prognosis

The prognosis of Myeloproliferative neoplasm is good with treatment. Without treatment, Myeloproliferative neoplasm may result in death. The 5- and 10-year survival rates for myeloproliferative neoplasm are 74% to 93% and 61% to 84%, respectively.

References

  1. 1.0 1.1 Ma X, Does M, Raza A, Mayne ST (2007). "Myelodysplastic syndromes: incidence and survival in the United States". Cancer. 109 (8): 1536–42. doi:10.1002/cncr.22570. PMID 17345612.
  2. 2.0 2.1 2.2 Agarwal MB, Malhotra H, Chakrabarti P, Varma N, Mathews V, Bhattacharyya J; et al. (2015). "Myeloproliferative neoplasms working group consensus recommendations for diagnosis and management of primary myelofibrosis, polycythemia vera, and essential thrombocythemia". Indian J Med Paediatr Oncol. 36 (1): 3–16. doi:10.4103/0971-5851.151770. PMC 4363847. PMID 25810569.
  3. DiNardo CD, Daver N, Jain N, Pemmaraju N, Bueso-Ramos C, Yin CC; et al. (2014). "Myelodysplastic/myeloproliferative neoplasms, unclassifiable (MDS/MPN, U): natural history and clinical outcome by treatment strategy". Leukemia. 28 (4): 958–61. doi:10.1038/leu.2014.8. PMC 3981947. PMID 24492324.
  4. Vannucchi AM (2017). "From leeches to personalized medicine: evolving concepts in the management of polycythemia vera". Haematologica. 102 (1): 18–29. doi:10.3324/haematol.2015.129155. PMC 5210229. PMID 27884974.
  5. Bittencourt RI, Vassallo J, Chauffaille Mde L, Xavier SG, Pagnano KB, Nascimento AC; et al. (2012). "Philadelphia-negative chronic myeloproliferative neoplasms". Rev Bras Hematol Hemoter. 34 (2): 140–9. doi:10.5581/1516-8484.20120034. PMC 3459391. PMID 23049404.
  6. Frewin, R (October 2012). "Headache in essential thrombocythaemia" (PDF). International Journal of Clinical Practice. 66 (10): 976–83. doi:10.1111/j.1742-1241.2012.02986.x. PMC 3469735. PMID 22889110. Unknown parameter |coauthors= ignored (help)