Volume 5, Number 9; February 06, 2006
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Volume 5, Number 9; February 06, 2006 |
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38-year-old African-American female with progressive loss of vision, eye pain, and chest pain.
Recommended reading:
Polycythemia vera: a comprehensive review and clinical recommendations.[see comment]. [Review]
Polycythemia vera: myths, mechanisms, and management. [Review]
A clinical update in polycythemia vera and essential thrombocythemia. [Review]
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Patient:
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Session Handout:
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Clinical Question: 1) What is polycythemia vera?
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Readings:
Link Directly to Fulltext article in Ovid <2> Unique Identifier [PMID]: 12583529 Authors: Tefferi A. Institution: Division of Hematology and Internal Medicine, Mayo Clinic, Rochester, Minn 55905, USA. tefferi.ayalew@mayo.edu Title: Polycythemia vera: a comprehensive review and clinical recommendations.[see comment]. [Review] [340 refs] Comments Comment in: Mayo Clin Proc. 2004 Mar;79(3):430; author reply 430-1; PMID: 15008617
Source: Mayo Clinic Proceedings. 78(2):174-94, 2003 Feb. Abstract: More than a century has elapsed since the appearance of the modern descriptions of polycythemia vera (PV). During this time, much has been learned regarding disease pathogenesis and PV-associated molecular aberrations. New information has allowed amendments to traditional diagnostic criteria. Phlebotomy remains the cornerstone treatment of PV, whereas myelosuppressive agents may augment the benefit of using phlebotomy for thrombosis prevention in high-risk patients. Excessive aspirin use is contraindicated in PV, although the use of lower-dose aspirin has been shown to be safe and effective in alleviating microvascular symptoms including erythromelalgia and headaches. Recent studies have shown the utility of selective serotonin receptor antagonists for treating PV-associated pruritus. Nevertheless, many questions remain unanswered. What is the specific genetic mutation or altered molecular pathway that is causally related to the disease? In the absence of a specific molecular marker, how is a working diagnosis of PV made? What evidence supports current practice in the management of PV? This article summarizes both old and new information on PV; proposes a modern diagnostic algorithm to formulate a working diagnosis; and provides recommendations for patient management, relying whenever possible on an evidence-based approach. [References: 340] Publication Type: Journal Article. Review.
Link Directly to Fulltext Article at Publisher <3> Unique Identifier [PMID]: 12393615 Authors: Spivak JL. Institution: Division of Hematology, Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA. jlspivak@jhmi.edu Title: Polycythemia vera: myths, mechanisms, and management. [Review] [369 refs]
Source: Blood. 100(13):4272-90, 2002 Dec 15. Publication Type: Journal Article. Review.
Link Directly to Fulltext Article at Science Direct <4> Unique Identifier [PMID]: 10967156 Authors: Tefferi A. Solberg LA. Silverstein MN. Institution: Division of Hematology and Internal Medicine (AT, MNS), Mayo Clinic and Mayo Foundation, Rochester, Minnesota, USA. Title: A clinical update in polycythemia vera and essential thrombocythemia. [Review] [97 refs]
Source: American Journal of Medicine. 109(2):141-9, 2000 Aug 1. Abstract: Polycythemia vera and essential thrombocythemia pose specific management issues that distinguish them from other chronic myeloproliferative disorders. They are associated with a better prognosis, as well as a variable risk of thrombohemorrhagic complications. In addition, essential thrombocythemia occurs comparatively more often in young people and women. Treatment strategies for patients with polycythemia vera and essential thrombocythemia must consider the possibility of long-term survival, morbidity from thrombotic complications, transformation into myelofibrosis with myeloid metaplasia or acute myeloid leukemia, and the effect of specific therapies on the incidence of leukemic transformation and on pregnancy. There is increasing concern about the possible leukemogenic effect of hydroxyurea. Newer therapeutic agents, including interferon alpha and anagrelide, are being used more often. Ongoing studies are reexamining the effects of low-dose aspirin in preventing thrombotic complications. [References: 97] Publication Type: Journal Article. Review.
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Resident Report / Department of Medicine & Grady Branch Library Emory University School of Medicine 2005 Edition Participating Faculty: Carlos Del Rio MD / Joyce Doyle MD / Lorenzo Difrancesco MD / Erich Folch MD / Alicia Hidron MD
Contact:
Karl Woodworth
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