Risk of leukemic transformation in PV and ET patients |
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| Authors: | Chomienne Christine,Rain Jean Didier,Brière Jean French PV ET Study Groups |
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| Affiliation: | 1. Service de Médecine Nucléaire, Unité de Biologie Cellulaire, Hôpital Saint Louis, 1, avenue Claude Vellefaux, Paris 75010, France;2. Hôpital Beaujon, 100 boulevard du Général Leclerc, Clichy 92110, France;1. Department of Medicine, University of Wisconsin, Madison, WI, USA;2. Department of Pathology and Laboratory Medicine, University of Wisconsin, Madison, WI, USA;3. Institute for Inflammation Research, Center for Rheumatology and Spine Diseases, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark;4. Institute for Molecules and Materials and Radboud Institute for Molecular Life Sciences, Radboud University, Nijmegen, The Netherlands;5. Department of Medical Microbiology and Immunology, University of Wisconsin-Madison, Madison, WI, USA;6. William S. Middleton Memorial Veterans Hospital, Madison, WI, USA;1. Hematology department, Hematology Unit, IRCCS Ca’ Granda Ospedale Maggiore Policlinico of Milan, Milan, Italy;2. Division of Hematology, Niguarda Hospital, Milan, Italy;3. Hematology department, Hematology Unit, IRCCS Ca’ Granda Ospedale Maggiore Policlinico and University of Milan, Milan, Italy;4. Bio-statistical department, Bio-statistical Unit, Regina Elena National Cancer Institute, Rome, Italy;5. Hematology department, Hematology Unit ASST Cremona, Milan, Italy;1. Division of Neuroradiology, Department of Radiology, VA Ann Arbor Health System, University of Michigan Health System, 1500 East Medical Center Drive, Ann Arbor, MI 48109, USA;2. BMT Program, Huntsman Cancer Center, University of Utah, 2000 Circle of Hope. Office #2151, Salt Lake City, UT 84112, USA;3. Department of Pharmacy, University of Michigan Health System, 1500 East Medical Center Drive, Ann Arbor, MI 48109, USA;4. Division of Neuroradiology, Department of Radiology, University of Michigan Health System, 1500 East Medical Center Drive, Ann Arbor, MI 48109, USA |
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| Abstract: | ![]()
Despite a prolonged survival of around 15 years linked to a prolonged complete remission induced by myelosuppression, myeloproliferative syndromes such as polycythemia vera (PV) and essential thrombosis (ET) remain at risk of lethal adverse affects such as thrombotic events and acute transformation. The major risk at diagnosis, in the absence of treatment, is essentially thrombosis. Different therapeutic trials have shown the necessity to maintain circulating blood cells (RBC and platelets counts) near normal levels to avoid thrombosis. Phlebotomies alone in PV lead in the long run to metaplasia and increased platelet counts and should only be kept for emergency cell count reduction. Myelosuppression is thus until recently the most widely accepted effective alternative. However, the effects of long term chronic administration of myelosuppresive agents needs to be analyzed and monitored as the biological changes which appear during the course of these diseases linked or not to the intrinsic clonal haematopoietic abnormality may lead to malignant transformation. Thus, alternative therapies need to be evaluated and predisposition factors taken in account. |
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