Abstract
Simple SummaryPatients with chronic myeloproliferative neoplasms (MPNs) such as polycythemia vera and essential thrombocythemia have an elevated risk of acute leukemia. Recently, it has been recognized that the risk of solid cancers is also increased. In the past decade, several studies have compared cancer frequency in patients with MPNs with the general population. In our study, we present results sampled from 12 previous studies, totaling more than 65,000 patients with MPNs identified through large registries. Patients with MPNs were compared to the age/sex-matched general population. Our results show that risk of new cancers is 1.5–3.0-fold elevated in patients with MPNs. In particular, lymphomas and cancers of the skin, lung, kidney, and thyroid gland occur more frequently. The difference in cancer occurrence is highest in the age group 60–79 years. Our results indicate that clinical follow up of patients with MPNs should include awareness of the increased cancer risk.In the past decade, several studies have reported that patients with chronic myeloproliferative neoplasms (MPNs) have an increased risk of second solid cancer or lymphoid hematological cancer. In this qualitative review study, we present results from studies that report on these cancer risks in comparison to cancer incidences in the general population or a control group. Our literature search identified 12 such studies published in the period 2009–2018 including analysis of more than 65,000 patients. The results showed that risk of solid cancer is 1.5- to 3.0-fold elevated and the risk of lymphoid hematological cancer is 2.5- to 3.5-fold elevated in patients with MPNs compared to the general population. These elevated risks apply to all MPN subtypes. For solid cancers, particularly risks of skin cancer, lung cancer, thyroid cancer, and kidney cancer are elevated. The largest difference in cancer risk between patients with MPN and the general population is seen in patients below 80 years. Cancer prognosis is negatively affected due to cardiovascular events, thrombosis, and infections by a concurrent MPN diagnosis mainly among patients with localized cancer. Our review emphasizes that clinicians caring for patients with MPNs should be aware of the very well-documented increased risk of second non-myeloid cancers.
Highlights
Myeloproliferative neoplasms (MPNs) encompassing essential thrombocythemia (ET), polycythemia vera (PV), primary myelofibrosis (PMF), chronic myeloid leukemia (CML), and unspecifiedCancers 2020, 12, 3061; doi:10.3390/cancers12103061 www.mdpi.com/journal/cancersMPNs are chronic stem cell cancers of the bone marrow [1]
Three studies identified and followed patients through the Swedish cancer registry that were diagnosed with MPN in the period 1958–2006 [22], 1973–2009 [11], or 1958–2015 [13] and some patients are likely to have been reported in multiple publications and analyses
Most of the studies reported on the observed number of patients with second cancer and compared this to the expected number based on the age/sex-standardized incidence ratio (SIR) or incidence rate ratios (IRRs) in the general population [10,11,13,22,23,24,25,26]
Summary
Myeloproliferative neoplasms (MPNs) encompassing essential thrombocythemia (ET), polycythemia vera (PV), primary myelofibrosis (PMF), chronic myeloid leukemia (CML), and unspecifiedCancers 2020, 12, 3061; doi:10.3390/cancers12103061 www.mdpi.com/journal/cancersMPNs are chronic stem cell cancers of the bone marrow [1]. Several large-scale registry studies have shown that solid cancers and hematological lymphoid malignancies occur more frequently in patients with MPNs compared to the general population [9,10,11,12,13]. The mechanisms behind this increased second cancer risk are elusive but an inherent tendency to cancer, shared risk factors, and anti-neoplastic treatment of MPNs may be involved [4,14,15,16,17,18,19,20,21].
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