Abstract
6621 Background: Essential thrombocythemia (ET) and polycythemia vera (PV) are chronic myeloproliferative neoplasms (MPN). Patients (pts) are at risk of thrombohemorrhagic complications for which they are often treated with different agents. Previous studies suggested that the risk of secondary malignancy (SM) may be increased in pts with PV or ET treated with Hydroxyurea (HU) or Alkylating agents (ALK). We report the incidence of SM in pts with ET and PV at our institution and treatment received. Methods: We performed a retrospective chart review of all pts with ET and PV seen at MD Anderson (diagnosis according to WHO) between January, 1960 and September 2010, recorded the pathological diagnosis of SM, and performed an analysis to look for differences in risk of SM according to treatment received. Results: A total of 437 pts charts (ET 263, PV 174) were evaluated. 29 pts developed SM [12 ET (4.6%), 17 PV (9.8%)] with median time to diagnosis of 68 months (14-167) in ET pts and 76 months (1-247) in PV pts. 6 pts developed 2 SM. 23 Pts had 29 solid tumors [7 Skin cancers (ET 2, PV 5), 5 breast (ET 2, PV3), 4 lung (ET 3, PV1), 4 genitourinary (ET 2, PV 2), 2 Thyroid (PV 2) and 7 other cancers]. 6 pts had hematologic malignancy [4 AML (ET 2, PV 2), 1 CLL (ET) and 1 NHL (ET)]. 341 (78%) of the 437 pts [222 (84%) ET; 119 (68%) PV] received one or combination of: 1- HU: 259 (60%) pts [162(62%) ET; 97 (56%) PV]; 2- Anagrelide (AG): 145 (33%) pts [112 (43%) ET; 33 (19%) PV]; 3- interferon (INF): 69 (16%) pts [47 (18%) ET; 22 (13%) PV]; 4- ALK (busulfan, melphalan or chlorambucil): 23 (5%) pts [16 (6%) ET; 7 (4%) PV]. Median follow up (MFU) was 69 months (1-396) with 27% of pts followed > 10 y. Risk of SM was 7% in pts treated with HU (ET 6%; PV 8%), 5% with AG (ET 4%; PV 9%), 4% with INF (ET 6%, PV 0%), and none of the pts receiving ALK developed SM (perhaps due to low number of pts and short MFU). 8 (8%) of the 96 pts not receiving any treatment developed SM. No statistical difference in SM risk was observed among treated and non treated groups. Conclusions: Our analysis confirmed inherited risk of developing SM in pts with ET and PV. Contrary to previous reports, the type of therapy received did not increase that risk, perhaps due to a relatively short MFU.
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