The coexistence or the development of Philadelphia chromosome-negative myeloproliferative neoplasms after a lymphoproliferative disease in the same patient is an extremely rare event. We report the case of a 72-year-old man who developed JAK2V617F polycythemia vera 3 years after the diagnosis and treatment of primary diffuse large B cell non-Hodgkin's lymphoma of the central nervous system. We also review the literature regarding the pathogenesis underlying the association of myeloproliferative and lymphoproliferative chronic disorders.

Vannucchi A, Masala G, Antonioli E, Susini MC, Guglielmelli P, Pieri L, Maggi L, Caini S, Palli D, Mogani C, Ponziani V, Pancrazi A, Annunziato F, Bosi A: Increased risk of lymphoid neoplasms in patients with Philadelphia chromosome-negative myeloproliferative neoplasms. Cancer Epidemiol Biomarkers Prev 2009;18:2068-2073.
Vainchenker W, Delhommeau F, Constantinescu SN, Bernard OA: New mutations and pathogenesis of myeloproliferative neoplasms. Blood 2011;118:1723-1735.
Passamonti F, Rumi E, Pungolino E, Malabarba L, Bertazzoni P, Valentini M, Orlandi E, Arcaini L, Brusamolino E, Pascutto C, Cazzola M, Morra E, Lazzarino M: Life expectancy and prognostic factors for survival in patients with polycythemia vera and essential thrombocythemia. Am J Med 2004;117:755-761.
Rumi E, Passamonti F, Chiara E, Pietra D, Arcaini L, Astori C, Zibellini S, Boveri E, Pascutto C, Lazzarino M: Increased risk of lymphoid neoplasm in patients with myeloproliferative neoplasm: a study of 1,915 patients. Haematologica 2011;96:454-458.
Bethard WF, Block MH, Robson M: Coexistent chronic lymphatic leukemia and polycythemia vera: morphologic and clinical studies with particular reference to unusual iron metabolism. Blood 1953;8:934-943.
Laurenti L, Tarnani M, Nichele I, Ciolli S, Cortelezzi A, Forconi F, Rossi D, Mauro FR, D'Arena G, Del Poeta G, Montanaro M, Morabito F, Musolino C, Callea V, Falchi L, Tedeschi A, Ambrosetti A, Gaidano G, Leone G, Foà R: The coexistence of chronic lymphocytic leukemia and myeloproliferative neoplasms: a retrospective multicentric GIMEMA experience. Am J Hematol 2011;86:1007-1012.
Carulli G, Test C, Azzarà A: Association between non-Hodgkin lymphoma and essential thrombocythemia. Haematologica 1997;82:382.
Rizzi R, Liso A, Pannunzio A, Carluccio P, Specchia G, Liso V: Concomitant primary polycythemia vera and follicle center cell non-Hodgkin lymphoma: a case report and review of the literature. Leuk Lymphoma 2002;43:2217-2220.
Palandri F, Derenzini E, Ottaviani E, Polverelli N, Catani L, Salmi F, Sabattini E, Bacci F, Zinzani PL, Baccarani M, Vianelli L: Association of essential thrombocythemia and non-Hodgkin lymphoma: a single-centre experience. Leuk Lymphoma 2009;50:481-484.
Fink L, Bauer F, Perry JJ: Coincidental polycythemia vera and multiple myeloma: case report and review. Am J Hematol 1993;44:196-200.
Lichtman SM, Wasil T, Ahmad M, Brody J: Development of essential thrombocythemia in a patient treated with interferon alfa and pentostatin for hairy cell leukemia. Leuk Lymphoma 1998;28:423-427.
Bosch F, Cervantes F, Lopez-Guillermo A, Carreras E, Montserrat E, Rozman C: Polycythaemia vera following non-Hodgkin's lymphoma. Leuk Lymphoma 1992;8:501-502.
Maciel JF, de Lourdes Chauffaille M, Inaoka RJ, Colleoni GW, Yamamoto M: Essential thrombocythemia after treatment of non-Hodgkin lymphoma. Leuk Res 2007;31:1593-1595.
Armitage JO, Carbona PP, Connors JM, Levina A, Bennet JM, Kroll S: Treatment-related myelodysplasia and acute leukemia in non-Hodgkin's lymphoma patients. J Clin Oncol 2003;5:897-906.
Antonioli E, Guglielmelli P, Poli G, Santini V, Bosi A, Vannucchi AM: Polycythemia vera following autologous transplantation for AML: insights on the kinetics of JAK2 V617F clonal dominance. Blood 2007;110:4620-4621.
Walker AR, Rothberg PG, Liesveld JL: A case of JAK2 positive essential thrombocythemia 16.5 years after autologous marrow transplantation for AML. Bone Marrow Transplant 2007;39:725-726.
Belotti A, Doni E, Elli E, Lanzi E, Pioltelli P, Pogliani EM: Development of polycythemia vera after chemotherapy-induced remission of acute myeloid leukemia: a case report. Acta Haematol 2011;126:52-53.
Plo I, Nakatake M, Malivert L, de Villartay JP, Giraudier S, Villeval JL, Wiesmuller L, Vainchenker W: JAK2 stimulates homologous recombination and genetic instability: potential implication in the heterogeneity of myeloproliferative disorders. Blood 2008;112:1402-1412.
Schlaifer D, Dastugue N, Brousset P, Delsol G, Muller C, Rigal-Huguet F, Attal M, Laurent G, Pris J: B-cell lymphoma following polycythemia vera: evidence for the involvement of two different clones. Leukemia 1994;8:895-896.
Arana-Yi C, Block AW, Sait SN, Ford LA, Barcos M, Baer MR: Therapy-related myelodysplastic syndrome and acute myeloid leukemia following treatment of acute myeloid leukemia: possible role of cytarabine. Leuk Res 2008;32:1043-1048.
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