From Vaquez to Dameshek through JAK…2 much for polycythemia vera to be feared?
Clinical practice in modern medicine is a gift that comes from afar. It comes sometimes from a lucky discovery, or sometimes from brave attempt chasing what at that time seemed crazy theories. Anyway, the gift we currently enjoy is the result of careful observation at first, and then like a sculptor with his chisel that free from marble an hidden figure, doctors and researchers carve during the years the features of many diseases.
In this number of the Internal and Emergency Medicine Journal, Landolfi and his co-workers  reviewed the principal characteristics of polycythemia vera (PV), starting from the history of the first published description by Luis Henry Vaquez in 1892 .
Since this first description something other than plethora and cyanosis were soon identified: the high risk of major thrombotic events potentially leading to death, both in arterial district (acute myocardial infarction, stroke or transient ischemic attack, TIA), and in venous district (deep vein thrombosis, p ...
- Landolfi R, Nicolazzi MA, Porfidia A, Di Gennaro L (2010) Polycythemia vera. Intern Emerg Med. doi:10.1007/s11739-010-0369-6
- Vaquez, H (1892) Sur une forme spéciale de cyanose s’acccompagnant d’hyperglobulie excessive et persistant. CR Soc Biol (Paris) 44: pp. 384-388
- Marchioli, R, Finazzi, G, Landolfi, R (2005) Vascular and neoplastic risk in a large color of patients with polycythemia vera. J Clin Oncol 23: pp. 2224-2232 CrossRef
- Prchal, JF, Axelrad, AA (1974) Letter: Bone marrow responses in polycythemia vera. N Engl J Med 290: pp. 1382
- Dameshek, W (1951) Some speculation on the myeloproliferative syndromes. Blood 6: pp. 372-375
- James, C, Ugo, V, Couedic, JP (2005) A unique clonal JAK2 mutation leading to constitutive signaling causes polycythemia vera. Nature 434: pp. 1144-1148 CrossRef
- Kralovics, R, Passamonti, F, Buser, AS (2005) A gain of function of JAK2 in myeloproliferative disorders. N Engl J Med 352: pp. 1779-1790 CrossRef
- Baxter, EJ, Scott, LM, Campbell, PJ, East, C, Fourouvlas, N, Swanton, S, Vassiliou, GS (2005) Acquired mutation of the tyrosine kinase JAK2 in human myeloproliferative disorders. Lancet 365: pp. 1054-1061
- Levine, RL, Wadleigh, M, Cools, J (2005) Activating mutation in the tyrosine kinase JAK2 in polycythemia vera, essential thrombocythemia and myeloid metaplasia with myelofibrosis. Cancer Cell 7: pp. 387-397 CrossRef
- James C (2008) The JAK2V617F mutation in polycythemia vera and other myeloproliferative disorders: one mutation for three diseases? Haematol Am Soc Hematol Educ Progr 69–75
- Ruggeri M, Finotto S, Fortuna S, Rodeghiero F (2010) Treatment outcome in a cohort of young patients with polycythemia vera. Intern Emerg Med. doi:10.1007/s11739-010-0429-y
- Passamonti, F, Malabarba, L, Orlandi, E (2003) Polycythemia vera in young patients: a study on long term risk of thrombosis, myelofibrosis and leukemia. Haematologica 88: pp. 13-18
- Landolfi, R, Marchioli, R, Kutti, J (2004) Efficacy and safety of low dose aspirin in polycythemia vera. N Engl J Med 350: pp. 114-124 CrossRef
- Reiter, A, Walz, C, Cross, NC (2007) Tyrosin kinase as therapeutic targets in BCR-ABL negative chronic myeloproliferative disorders. Curr Drug Targets 8: pp. 205-216 CrossRef
- Gaikwad, A, Vertovsek, S, Yoon, D (2007) Imatinib effect on growth and signal transduction in polycythemia vera. Exp Hematol 35: pp. 931-938 CrossRef
- From Vaquez to Dameshek through JAK…2 much for polycythemia vera to be feared?
Internal and Emergency Medicine
Volume 5, Issue 5 , pp 371-373
- Cover Date
- Print ISSN
- Online ISSN
- Springer Milan
- Additional Links