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Myeloproliferative neoplasms (MPN) are a group of hematological malignancies characterized by clonal proliferation of bone marrow stem cells and are classified into subgroups, including polycythemia vera, essential thrombocythemia (ET), primary myelofibrosis, chronic myeloid leukemia, and MPN unclassifiable. While the risk of developing acute myeloid leukemia is well known in patients with MPN, over the past 10 years, various studies have also reported on the increased risk of other cancers. Mette Brabrand and Henrik Frederiksen recently published a qualitative review, including 12 of these studies, which was published in the journal Cancers and is summarized below.
Figure 1. Screening and eligibility of articles for inclusion1
Table 1. SIR and IRR ranges for secondary malignancies in patients with MPN1
CLL, chronic lymphocytic leukemia; HL, Hodgkin lymphoma; IRR, incidence rate ratio; MM, multiple myeloma; NA, not available; NHL, non-Hodgkin lymphoma; NMSC, non-melanoma skin cancer; MPN, myeloproliferative neoplasms; SIR, standardized incidence ratio. |
||||
Type of solid cancer |
SIR range |
Publication no(s). |
IRR range |
Publication no(s). |
---|---|---|---|---|
All solid cancer |
0.8–5.2 |
3, 5, 6, 8, 10 |
1.4–1.6 |
7 |
Colon cancer |
0.6–1.6 |
3, 4, 5, 6, 8, 9, 10 |
0.9–1.0 |
7, 11 |
Lung cancer |
0.9–1.9 |
3, 4, 5, 6, 8, 9, 10 |
1.4–1.6 |
7, 11 |
Breast cancer |
0.8–1.3 |
3, 4, 5, 6, 8, 9, 10 |
1.0–1.1 |
7, 11 |
Melanoma |
1.7–3.7 |
3, 4, 5, 6, 9, 10 |
1.8–2.2 |
7, 11 |
NMSC |
1.0–3.3 |
3, 4, 5, 10 |
2.0 |
11 |
Kidney cancer |
1.1–2.4 |
3, 4, 5, 6, 8, 10 |
2.0–2.1 |
7, 11 |
Prostate cancer |
0.7–1.3 |
3, 4, 5, 6, 8, 9, 10 |
0.6–1.1 |
7, 11 |
Thyroid cancer |
0.5–3.7 |
4, 6, 9 |
2.3 |
7 |
Type of hematological cancer |
SIR range |
Publication no(s). |
IRR range |
Publication no(s). |
All NHL |
1.1–9.7 |
1, 3, 4, 6, 9 |
1.6–2.3 |
7, 11 |
CLL |
12.4 |
1 |
2.5 |
7 |
MM |
1.6 |
4 |
1.6 |
7, 11 |
HL |
NA |
— |
2.8–3.1 |
7, 11 |
Patients with MPN have an increased risk of developing secondary cancers of the skin, lung, kidney, and thyroid, as well as lymphoid cancers. This may partly be due to the treatment that patients receive, as hydroxyurea has been shown to increase the risk of non-melanoma skin cancers. Additionally, low-dose aspirin, which is used to prevent thrombosis in patients with MPN, is associated with a decreased risk of colorectal cancer, possibly because of an increased risk of gastrointestinal bleeding and therefore detection and removal of colorectal polyps.
The elevated risk of lung cancer in patients with MPN may be biased as blood test results of smokers and patients with lung disease can resemble those of patients with untreated MPN, such as high hematocrit, thrombocytosis, and leukocytosis. Therefore, these patients could be mistakenly registered with an MPN diagnosis. Nevertheless, smoking has been shown to be associated with an increased risk of developing MPN.
Age is another factor that modifies the cancer risk uniformly across studies, with the largest elevated risk increase seen for patients between 60 and 79 years. Detection bias, due to routine surveillance of patients with MPN may have influenced these results to some degree. However, as the incidence of major cancer types, such as colorectal cancer, breast cancer, and prostate cancer, were not increased among patients with MPN, detection bias is unlikely to explain the observed increased risks in specific cancer types, such as lymphoid malignancies. Although cancer surveillance among all patients with MPN may be unnecessary, it may be beneficial for those at increased risk, such as the 60–70 age group.
Table 2. Publications comparing the incidence of SMs in patients with MPN1
CLL, chronic lymphocytic leukemia; CML, chronic myeloid leukemia; ET, essential thrombocythemia; HL, Hodgkin lymphoma; MF, myelofibrosis; MM, multiple myeloma; MPN, myeloproliferative neoplasms; MPN-NOS, MPN-not otherwise specified; MPN-U, MPN-unspecified; NHL, non-Hodgkin lymphoma; PMF, primary myelofibrosis; PV, polycythemia vera; SM, secondary malignancy; SMF, secondary myelofibrosis. |
|||||
Number |
Publication |
Patient no. |
MPN subtype |
Median years of follow-up |
Type of SM assessed |
---|---|---|---|---|---|
1 |
Vannuchi, 2009 |
820 |
ET (57%), PV (43%) |
3.3 |
NHL, CLL |
2 |
Rumi, 2011 |
1,915 |
ET (44%), PV (34%), MF (18%), SMF (4%) |
5.2 |
— |
3 |
Frederiksen, 2011 |
7,229 |
ET (22%), PV (63%), CML (14%) |
ET, 4.0; PV, 5.0; CML, 2.4 |
NHL |
4 |
Fallah, 2011 |
3,530 |
PV |
— |
NHL, MM |
5 |
Susini, 2012 |
733 |
ET (51%), PV (41%), PMF (8%) |
Mean 6.5 |
— |
6 |
Khanal, 2015 |
3,941 |
PV |
4.7 |
NHL |
7 |
Brunner, 2016 |
20,250 |
ET, PV, MF, MPN-U |
3.5 |
NHL, HL, CLL, MM |
8 |
Shrestha, 2016 |
8,116 |
ET |
3.0 |
Lymphoma |
9 |
Masarova, 2016 |
417 |
ET (40%), PV (60%) |
— |
NHL |
10 |
Landtblom, 2018 |
9,379 |
ET (28%), PV (45%), PMF (15%), MPN-NOS (12%) |
7.7 |
Lymphoma, MM |
11 |
Chattopadhyay, 2018 |
13,805 |
ET (30%), PV (48%), MF (11%), MPN-NOS (12%) |
ET, 4; PV, 6; MF, 2; MPN-U. 3 |
NHL, HL, MM |
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