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20+ yr without leukemic or fibrotic transformation in essential thrombocythemia or polycythemia vera: predictors at diagnosis

Objectives:  The current study identified patients with either essential thrombocythemia (ET) or polycythemia vera (PV) who have survived for at least 20 yr without the development of either acute myeloid leukemia/myelodysplastic syndrome (AML/MDS) or myelofibrosis (MF) and compared their presenting...

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Published in:European journal of haematology 2008-05, Vol.80 (5), p.386-390
Main Authors: Tefferi, Ayalew, Gangat, Naseema, Wolanskyj, Alexandra P., Schwager, Susan, Pardanani, Animesh, Lasho, Terra L., Mesa, Ruben, McClure, Rebecca F., Li, Chin-Yang, Hanson, Curtis A.
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cited_by cdi_FETCH-LOGICAL-c4058-e335c1ca53259fd37885c3627cc67ce1377ea4e824063b2c2259a1c406226aa53
cites cdi_FETCH-LOGICAL-c4058-e335c1ca53259fd37885c3627cc67ce1377ea4e824063b2c2259a1c406226aa53
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container_issue 5
container_start_page 386
container_title European journal of haematology
container_volume 80
creator Tefferi, Ayalew
Gangat, Naseema
Wolanskyj, Alexandra P.
Schwager, Susan
Pardanani, Animesh
Lasho, Terra L.
Mesa, Ruben
McClure, Rebecca F.
Li, Chin-Yang
Hanson, Curtis A.
description Objectives:  The current study identified patients with either essential thrombocythemia (ET) or polycythemia vera (PV) who have survived for at least 20 yr without the development of either acute myeloid leukemia/myelodysplastic syndrome (AML/MDS) or myelofibrosis (MF) and compared their presenting features with those in whom these complications occurred in the first 10 yr of disease. Methods:  The study patients were selected from an institutional database of 1061 patients with either ET (n = 603) or PV (n = 458). In both instances, three distinct groups were delineated and their presenting features compared; group A included patients who have remained AML/MDS/MF free after a minimum follow‐up of 20 yr; groups B and C included patients who developed either AML/MDS or MF, respectively, in the first decade of their disease. Results:  The respective number of patients who fulfilled the above‐mentioned criteria for inclusion in groups A, B and C were 40, 12 and 8 for ET and 23, 18 and 12 for PV. In ET, compared with both groups B and C, group A displayed significantly fewer patients with less than normal hemoglobin level (P 
doi_str_mv 10.1111/j.1600-0609.2008.01038.x
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Methods:  The study patients were selected from an institutional database of 1061 patients with either ET (n = 603) or PV (n = 458). In both instances, three distinct groups were delineated and their presenting features compared; group A included patients who have remained AML/MDS/MF free after a minimum follow‐up of 20 yr; groups B and C included patients who developed either AML/MDS or MF, respectively, in the first decade of their disease. Results:  The respective number of patients who fulfilled the above‐mentioned criteria for inclusion in groups A, B and C were 40, 12 and 8 for ET and 23, 18 and 12 for PV. In ET, compared with both groups B and C, group A displayed significantly fewer patients with less than normal hemoglobin level (P &lt; 0.0001 and =0.02) or male sex (P = 0.005 and 0.05), respectively. On multivariable analysis, only anemia sustained its significance. A similar analysis in PV revealed an association between group B and leukocytosis using a leukocyte count threshold of either 10 or 15 × 109/L (P = 0.02). Conclusion:  The current study identifies PV patients with leukocytosis and ET patients with anemia as the most likely to undergo leukemic or fibrotic transformation.</description><identifier>ISSN: 0902-4441</identifier><identifier>EISSN: 1600-0609</identifier><identifier>DOI: 10.1111/j.1600-0609.2008.01038.x</identifier><identifier>PMID: 18221390</identifier><language>eng</language><publisher>Oxford, UK: Blackwell Publishing Ltd</publisher><subject>Adult ; Aged ; Aged, 80 and over ; Female ; Fibrosis - pathology ; Humans ; JAK2 ; JAK2V617F ; leukemia ; Leukemia - pathology ; Male ; Middle Aged ; Polycythemia Vera - diagnosis ; Polycythemia Vera - epidemiology ; prognosis ; survival ; Thrombocythemia, Essential - diagnosis ; Thrombocythemia, Essential - epidemiology ; Time Factors ; V617F</subject><ispartof>European journal of haematology, 2008-05, Vol.80 (5), p.386-390</ispartof><rights>2008 The Authors</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c4058-e335c1ca53259fd37885c3627cc67ce1377ea4e824063b2c2259a1c406226aa53</citedby><cites>FETCH-LOGICAL-c4058-e335c1ca53259fd37885c3627cc67ce1377ea4e824063b2c2259a1c406226aa53</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,776,780,27901,27902</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/18221390$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Tefferi, Ayalew</creatorcontrib><creatorcontrib>Gangat, Naseema</creatorcontrib><creatorcontrib>Wolanskyj, Alexandra P.</creatorcontrib><creatorcontrib>Schwager, Susan</creatorcontrib><creatorcontrib>Pardanani, Animesh</creatorcontrib><creatorcontrib>Lasho, Terra L.</creatorcontrib><creatorcontrib>Mesa, Ruben</creatorcontrib><creatorcontrib>McClure, Rebecca F.</creatorcontrib><creatorcontrib>Li, Chin-Yang</creatorcontrib><creatorcontrib>Hanson, Curtis A.</creatorcontrib><title>20+ yr without leukemic or fibrotic transformation in essential thrombocythemia or polycythemia vera: predictors at diagnosis</title><title>European journal of haematology</title><addtitle>Eur J Haematol</addtitle><description>Objectives:  The current study identified patients with either essential thrombocythemia (ET) or polycythemia vera (PV) who have survived for at least 20 yr without the development of either acute myeloid leukemia/myelodysplastic syndrome (AML/MDS) or myelofibrosis (MF) and compared their presenting features with those in whom these complications occurred in the first 10 yr of disease. Methods:  The study patients were selected from an institutional database of 1061 patients with either ET (n = 603) or PV (n = 458). In both instances, three distinct groups were delineated and their presenting features compared; group A included patients who have remained AML/MDS/MF free after a minimum follow‐up of 20 yr; groups B and C included patients who developed either AML/MDS or MF, respectively, in the first decade of their disease. Results:  The respective number of patients who fulfilled the above‐mentioned criteria for inclusion in groups A, B and C were 40, 12 and 8 for ET and 23, 18 and 12 for PV. In ET, compared with both groups B and C, group A displayed significantly fewer patients with less than normal hemoglobin level (P &lt; 0.0001 and =0.02) or male sex (P = 0.005 and 0.05), respectively. On multivariable analysis, only anemia sustained its significance. A similar analysis in PV revealed an association between group B and leukocytosis using a leukocyte count threshold of either 10 or 15 × 109/L (P = 0.02). Conclusion:  The current study identifies PV patients with leukocytosis and ET patients with anemia as the most likely to undergo leukemic or fibrotic transformation.</description><subject>Adult</subject><subject>Aged</subject><subject>Aged, 80 and over</subject><subject>Female</subject><subject>Fibrosis - pathology</subject><subject>Humans</subject><subject>JAK2</subject><subject>JAK2V617F</subject><subject>leukemia</subject><subject>Leukemia - pathology</subject><subject>Male</subject><subject>Middle Aged</subject><subject>Polycythemia Vera - diagnosis</subject><subject>Polycythemia Vera - epidemiology</subject><subject>prognosis</subject><subject>survival</subject><subject>Thrombocythemia, Essential - diagnosis</subject><subject>Thrombocythemia, Essential - epidemiology</subject><subject>Time Factors</subject><subject>V617F</subject><issn>0902-4441</issn><issn>1600-0609</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2008</creationdate><recordtype>article</recordtype><recordid>eNqNkM-O0zAQhy0EYkvhFZBPXFCyYzuxEyQOaLW0i1b8kYDlZrmuQ91N4mI7bHMCiTflSXBoVa744hnN75uRPoQwgZykd77NCQfIgEOdU4AqBwKsyvf30Ow0uI9mUAPNiqIgZ-hRCFsAoDURD9EZqSglrIYZ-kHh-e-fv0aP72zcuCHi1gy3prMaO48bu_Iupjp61YfG-U5F63pse2xCMH20qsVx4123cnqMm8Spidu5djz1341XL_DOm7XV0fmAVcRrq772LtjwGD1oVBvMk-M_R59eX368WGbX7xZXF6-uM11AWWWGsVITrUpGy7pZM1FVpWacCq250IYwIYwqTEUL4GxFNU0xRRLLKeUqYXP07LB35923wYQoOxu0aVvVGzcEKaCoOUnwHFWHoPYuBG8aufO2U36UBOQkX27l5FhOjuUkX_6VL_cJfXq8Maw6s_4HHm2nwMtD4M62ZvzvxfLyzXKqEp8deBui2Z945W8lF0yU8ubtQt4s3_PFB_5ZfmF_AGYWpN8</recordid><startdate>200805</startdate><enddate>200805</enddate><creator>Tefferi, Ayalew</creator><creator>Gangat, Naseema</creator><creator>Wolanskyj, Alexandra P.</creator><creator>Schwager, Susan</creator><creator>Pardanani, Animesh</creator><creator>Lasho, Terra L.</creator><creator>Mesa, Ruben</creator><creator>McClure, Rebecca F.</creator><creator>Li, Chin-Yang</creator><creator>Hanson, Curtis A.</creator><general>Blackwell Publishing Ltd</general><scope>BSCLL</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope></search><sort><creationdate>200805</creationdate><title>20+ yr without leukemic or fibrotic transformation in essential thrombocythemia or polycythemia vera: predictors at diagnosis</title><author>Tefferi, Ayalew ; Gangat, Naseema ; Wolanskyj, Alexandra P. ; Schwager, Susan ; Pardanani, Animesh ; Lasho, Terra L. ; Mesa, Ruben ; McClure, Rebecca F. ; Li, Chin-Yang ; Hanson, Curtis A.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c4058-e335c1ca53259fd37885c3627cc67ce1377ea4e824063b2c2259a1c406226aa53</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2008</creationdate><topic>Adult</topic><topic>Aged</topic><topic>Aged, 80 and over</topic><topic>Female</topic><topic>Fibrosis - pathology</topic><topic>Humans</topic><topic>JAK2</topic><topic>JAK2V617F</topic><topic>leukemia</topic><topic>Leukemia - pathology</topic><topic>Male</topic><topic>Middle Aged</topic><topic>Polycythemia Vera - diagnosis</topic><topic>Polycythemia Vera - epidemiology</topic><topic>prognosis</topic><topic>survival</topic><topic>Thrombocythemia, Essential - diagnosis</topic><topic>Thrombocythemia, Essential - epidemiology</topic><topic>Time Factors</topic><topic>V617F</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Tefferi, Ayalew</creatorcontrib><creatorcontrib>Gangat, Naseema</creatorcontrib><creatorcontrib>Wolanskyj, Alexandra P.</creatorcontrib><creatorcontrib>Schwager, Susan</creatorcontrib><creatorcontrib>Pardanani, Animesh</creatorcontrib><creatorcontrib>Lasho, Terra L.</creatorcontrib><creatorcontrib>Mesa, Ruben</creatorcontrib><creatorcontrib>McClure, Rebecca F.</creatorcontrib><creatorcontrib>Li, Chin-Yang</creatorcontrib><creatorcontrib>Hanson, Curtis A.</creatorcontrib><collection>Istex</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>European journal of haematology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Tefferi, Ayalew</au><au>Gangat, Naseema</au><au>Wolanskyj, Alexandra P.</au><au>Schwager, Susan</au><au>Pardanani, Animesh</au><au>Lasho, Terra L.</au><au>Mesa, Ruben</au><au>McClure, Rebecca F.</au><au>Li, Chin-Yang</au><au>Hanson, Curtis A.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>20+ yr without leukemic or fibrotic transformation in essential thrombocythemia or polycythemia vera: predictors at diagnosis</atitle><jtitle>European journal of haematology</jtitle><addtitle>Eur J Haematol</addtitle><date>2008-05</date><risdate>2008</risdate><volume>80</volume><issue>5</issue><spage>386</spage><epage>390</epage><pages>386-390</pages><issn>0902-4441</issn><eissn>1600-0609</eissn><abstract>Objectives:  The current study identified patients with either essential thrombocythemia (ET) or polycythemia vera (PV) who have survived for at least 20 yr without the development of either acute myeloid leukemia/myelodysplastic syndrome (AML/MDS) or myelofibrosis (MF) and compared their presenting features with those in whom these complications occurred in the first 10 yr of disease. Methods:  The study patients were selected from an institutional database of 1061 patients with either ET (n = 603) or PV (n = 458). In both instances, three distinct groups were delineated and their presenting features compared; group A included patients who have remained AML/MDS/MF free after a minimum follow‐up of 20 yr; groups B and C included patients who developed either AML/MDS or MF, respectively, in the first decade of their disease. Results:  The respective number of patients who fulfilled the above‐mentioned criteria for inclusion in groups A, B and C were 40, 12 and 8 for ET and 23, 18 and 12 for PV. In ET, compared with both groups B and C, group A displayed significantly fewer patients with less than normal hemoglobin level (P &lt; 0.0001 and =0.02) or male sex (P = 0.005 and 0.05), respectively. On multivariable analysis, only anemia sustained its significance. A similar analysis in PV revealed an association between group B and leukocytosis using a leukocyte count threshold of either 10 or 15 × 109/L (P = 0.02). Conclusion:  The current study identifies PV patients with leukocytosis and ET patients with anemia as the most likely to undergo leukemic or fibrotic transformation.</abstract><cop>Oxford, UK</cop><pub>Blackwell Publishing Ltd</pub><pmid>18221390</pmid><doi>10.1111/j.1600-0609.2008.01038.x</doi><tpages>5</tpages></addata></record>
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identifier ISSN: 0902-4441
ispartof European journal of haematology, 2008-05, Vol.80 (5), p.386-390
issn 0902-4441
1600-0609
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source Wiley-Blackwell Read & Publish Collection
subjects Adult
Aged
Aged, 80 and over
Female
Fibrosis - pathology
Humans
JAK2
JAK2V617F
leukemia
Leukemia - pathology
Male
Middle Aged
Polycythemia Vera - diagnosis
Polycythemia Vera - epidemiology
prognosis
survival
Thrombocythemia, Essential - diagnosis
Thrombocythemia, Essential - epidemiology
Time Factors
V617F
title 20+ yr without leukemic or fibrotic transformation in essential thrombocythemia or polycythemia vera: predictors at diagnosis
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