Abstract
Aim of this study is to explore the role of different treatments on the development of secondary malignancies (SMs) in a large cohort of essential thrombocythemia (ET) patients. We report the experience of a regional cooperative group in a real‐life cohort of 1026 patients with ET. We divided our population into five different groups: group 0, no treatment; group 1, hydroxyurea (HU); group 2, alkylating agents (ALK); group 3, ALK + HU sequentially or in combination; and group 4, anagrelide (ANA) and/or α‐interferon (IFN) only. Patients from groups 1, 2, and 3 could also have been treated either with ANA and/or IFN in their medical history, considering these drugs not to have an additional cytotoxic potential. In all, 63 of the 1026 patients (6%) developed 64 SM during the follow‐up, after a median time of 50 months (range: 2–158) from diagnosis. In univariate analysis, a statistically significant difference was found only for gender (P = 0.035) and age (P = 0.0001). In multivariate analysis, a statistically significant difference was maintained for both gender and age (gender HR1.7 [CI 95% 1.037–2.818] P = 0.035; age HR 4.190 [CI 95% 2.308–7.607] P = 0.0001). The impact of different treatments on SMs development was not statistically significant. In our series of 1026 ET patients, diagnosed and followed during a 30‐year period, the different therapies administered, comprising HU and ALK, do not appear to have impacted on the development of SM. A similar rate of SMs was observed also in untreated patients. The only two variables which showed a statistical significance were male gender and age >60 years.
Highlights
Essential thrombocythemia (ET) is a myeloproliferative neoplasm, mostly occurring in elderly patients, frequently associated with major and minor vascular complications that cause increased morbidity and sometimes fatal complications [1,2,3]
Aim of this study is to explore the role of different treatments on the development of secondary malignancies (SMs) in a large cohort of essential thrombocythemia (ET) patients
We did not consider the development of acute leukemia (AL) as a SM because this event could be considered as part of the natural evolution of ET; we excluded the basaliomas because they cannot be considered real neoplasms and because it is well known that the use of hydroxyurea can cause this kind of skin tumors
Summary
Essential thrombocythemia (ET) is a myeloproliferative neoplasm, mostly occurring in elderly patients, frequently associated with major and minor vascular complications that cause increased morbidity and sometimes fatal complications [1,2,3]. Survival of ET patients during the first 10 years of the disease is similar to that of the general population of the same age. Cytoreduction is indicated in patients above 60 years of age or in those with previous thrombosis or a platelet count in excess of 1500 × 109/L [7, 8]. In these cases, hydroxyurea has emerged as the treatment of choice on account of its efficacy in reducing thrombotic complications [9]. Other cytoreductive and/or immune modulating drugs, including alkylating agents, α-interferon, and anagrelide, have been used and are still indicated in high-risk patients [7, 8]
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