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, weigh loss, and fatigue. Without treatment, the patient will develop symptoms of fever, abdominal pain, and bruising which may eventually lead to death.[1][2]
Complications
Myeloproliferative neoplasm may lead to the following complications:[3][2]
- [[thrombosis|Arterial thrombosis
- Venous thrombosis
- Spleen
- Splenomegaly
- Splenic infarction
- Splenic Abscess
- Fatigue
- Anemia
- Weight loss
- Leukocytosis
Prognosis
When properly diagnosed and carefully managed, some patients may survive for many years, depending on the specific disease they have. Recent advancements in CML treatment such as IFN alpha, bone marrow transplantation and recent development of an agent that inhibits the tyrosine kinase function of the BCR-ABL fusion protein, have substantially increased the life expectancy for patients with CML, so the median survival rates of 5-7 years are commonly reported. If similar mechanisms of pathogenesis do exist in the other MPNs, tyrosine kinase inhibitors or similar agents may significantly improve survival.
For polycythemia vera and essential thrombocytopenia, overall life expectancy may not be severely shortened, in view of the fact that they occur most frequently in older individuals.
If left untreated, patients with MPNs may die within months after the onset of symptoms.
References
- ↑ 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.
- ↑ 2.0 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.
- ↑ 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.