{"id":"https://openalex.org/W27612425","doi":null,"title":"Polycythemia vera and essential thrombocythemia in young patients.","display_name":"Polycythemia vera and essential thrombocythemia in young patients.","publication_year":1993,"publication_date":"1993-11-01","ids":{"openalex":"https://openalex.org/W27612425","mag":"27612425","pmid":"https://pubmed.ncbi.nlm.nih.gov/8039752"},"language":"en","primary_location":{"id":"pmid:8039752","doi":null,"is_oa":false,"landing_page_url":"https://pubmed.ncbi.nlm.nih.gov/8039752","pdf_url":null,"source":{"id":"https://openalex.org/S4306525036","display_name":"PubMed","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I1299303238","host_organization_name":"National Institutes of Health","host_organization_lineage":["https://openalex.org/I1299303238"],"host_organization_lineage_names":[],"type":"repository"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Haematologica","raw_type":"Journal Article"},"volume":null,"type":"article","is_traditional":true,"indexed_in":["pubmed"],"open_access":{"is_oa":false,"oa_status":"closed","oa_url":null,"any_repository_has_fulltext":false},"authorships":[{"author_position":"first","author":{"id":"https://openalex.org/A5082819090","display_name":"Maurizio Frezzato","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I4210152245","display_name":"Ospedale San Bortolo","ror":"https://ror.org/05wd86d64","country_code":"IT","type":"healthcare","lineage":["https://openalex.org/I4210152245"]}],"countries":["IT"],"is_corresponding":false,"raw_author_name":"M Frezzato","raw_affiliation_strings":["Department of Hematology, San Bortolo Hospital, Vicenza, Italy"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Hematology, San Bortolo Hospital, Vicenza, Italy","institution_ids":["https://openalex.org/I4210152245"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5075657251","display_name":"Marco Ruggeri","orcid":"https://orcid.org/0000-0003-1557-0957","observed_orcids":["https://orcid.org/0000-0003-1557-0957"]},"institutions":[],"countries":[],"is_corresponding":false,"raw_author_name":"M Ruggeri","raw_affiliation_strings":[],"raw_orcid":null,"affiliations":[]},{"author_position":"middle","author":{"id":"https://openalex.org/A5006881585","display_name":"Giancarlo Castaman","orcid":"https://orcid.org/0000-0003-4973-1317","observed_orcids":["https://orcid.org/0000-0003-4973-1317"]},"institutions":[],"countries":[],"is_corresponding":false,"raw_author_name":"G Castaman","raw_affiliation_strings":[],"raw_orcid":null,"affiliations":[]},{"author_position":"last","author":{"id":"https://openalex.org/A5013529815","display_name":"Francesco Rodeghiero","orcid":"https://orcid.org/0000-0003-2253-2502","observed_orcids":["https://orcid.org/0000-0003-2253-2502"]},"institutions":[],"countries":[],"is_corresponding":false,"raw_author_name":"F Rodeghiero","raw_affiliation_strings":[],"raw_orcid":null,"affiliations":[]}],"institutions":[],"countries_distinct_count":1,"institutions_distinct_count":1,"corresponding_author_ids":[],"corresponding_institution_ids":[],"apc_list":null,"apc_paid":null,"fwci":0.2391,"has_fulltext":false,"cited_by_count":30,"citation_normalized_percentile":{"value":0.48214847,"is_in_top_1_percent":false,"is_in_top_10_percent":false},"cited_by_percentile_year":{"min":88,"max":96},"biblio":{"volume":"78","issue":"6 Suppl 2","first_page":"11","last_page":"7"},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T11628","display_name":"Myeloproliferative Neoplasms: Diagnosis and Treatment","score":1.0,"subfield":{"id":"https://openalex.org/subfields/2720","display_name":"Hematology"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},"topics":[{"id":"https://openalex.org/T11628","display_name":"Myeloproliferative Neoplasms: Diagnosis and Treatment","score":1.0,"subfield":{"id":"https://openalex.org/subfields/2720","display_name":"Hematology"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},{"id":"https://openalex.org/T10746","display_name":"Platelet Disorders and Treatments","score":0.0,"subfield":{"id":"https://openalex.org/subfields/2720","display_name":"Hematology"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},{"id":"https://openalex.org/T13374","display_name":"Hematological disorders and diagnostics","score":0.0,"subfield":{"id":"https://openalex.org/subfields/2720","display_name":"Hematology"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}}],"keywords":[{"id":"https://openalex.org/keywords/polycythemia-vera","display_name":"polycythemia vera","score":0.9990000128746033},{"id":"https://openalex.org/keywords/essential-thrombocythemia","display_name":"essential thrombocythemia","score":1.0},{"id":"https://openalex.org/keywords/young-patients","display_name":"young patients","score":0.9710000157356262},{"id":"https://openalex.org/keywords/thrombotic-complications","display_name":"thrombotic complications","score":0.8920000195503235},{"id":"https://openalex.org/keywords/bleeding-complications","display_name":"bleeding complications","score":0.996999979019165},{"id":"https://openalex.org/keywords/long-term-outcomes","display_name":"long-term outcomes","score":0.7289999723434448},{"id":"https://openalex.org/keywords/leukemic-transformation","display_name":"leukemic transformation","score":0.6959999799728394},{"id":"https://openalex.org/keywords/prognosis","display_name":"prognosis","score":0.6159999966621399},{"id":"https://openalex.org/keywords/natural-history-disease","display_name":"natural history (disease)","score":0.5260000228881836},{"id":"https://openalex.org/keywords/platelet-count","display_name":"platelet count","score":0.46700000762939453},{"id":"https://openalex.org/keywords/mortality","display_name":"mortality","score":0.4449999928474426},{"id":"https://openalex.org/keywords/acute-myeloid-leukemia","display_name":"acute myeloid leukemia","score":0.7630000114440918},{"id":"https://openalex.org/keywords/thrombosis","display_name":"thrombosis","score":0.37599998712539673},{"id":"https://openalex.org/keywords/myeloproliferative-neoplasms","display_name":"myeloproliferative neoplasms","score":0.8610000014305115}],"concepts":[{"id":"https://openalex.org/C71924100","wikidata":"https://www.wikidata.org/wiki/Q11190","display_name":"Medicine","level":0,"score":0.9067609906196594},{"id":"https://openalex.org/C2781057849","wikidata":"https://www.wikidata.org/wiki/Q1368780","display_name":"Essential thrombocythemia","level":3,"score":0.8773602843284607},{"id":"https://openalex.org/C2778837598","wikidata":"https://www.wikidata.org/wiki/Q948318","display_name":"Polycythemia vera","level":2,"score":0.8457719087600708},{"id":"https://openalex.org/C126322002","wikidata":"https://www.wikidata.org/wiki/Q11180","display_name":"Internal medicine","level":1,"score":0.6937903165817261},{"id":"https://openalex.org/C61511704","wikidata":"https://www.wikidata.org/wiki/Q1671857","display_name":"Incidence (geometry)","level":2,"score":0.6119793057441711},{"id":"https://openalex.org/C72563966","wikidata":"https://www.wikidata.org/wiki/Q1303415","display_name":"Cohort","level":2,"score":0.5976746082305908},{"id":"https://openalex.org/C90924648","wikidata":"https://www.wikidata.org/wiki/Q120569","display_name":"Gastroenterology","level":1,"score":0.5428140759468079},{"id":"https://openalex.org/C141071460","wikidata":"https://www.wikidata.org/wiki/Q40821","display_name":"Surgery","level":1,"score":0.39531996846199036},{"id":"https://openalex.org/C121332964","wikidata":"https://www.wikidata.org/wiki/Q413","display_name":"Physics","level":0,"score":0.0},{"id":"https://openalex.org/C120665830","wikidata":"https://www.wikidata.org/wiki/Q14620","display_name":"Optics","level":1,"score":0.0}],"mesh":[{"descriptor_ui":"D000206","descriptor_name":"Actuarial Analysis","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D000293","descriptor_name":"Adolescent","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D000328","descriptor_name":"Adult","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D002423","descriptor_name":"Cause of Death","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D002648","descriptor_name":"Child","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D003131","descriptor_name":"Combined Modality Therapy","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D005260","descriptor_name":"Female","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D006470","descriptor_name":"Hemorrhage","qualifier_ui":"Q000209","qualifier_name":"etiology","is_major_topic":false},{"descriptor_ui":"D006470","descriptor_name":"Hemorrhage","qualifier_ui":"Q000453","qualifier_name":"epidemiology","is_major_topic":false},{"descriptor_ui":"D006470","descriptor_name":"Hemorrhage","qualifier_ui":"Q000517","qualifier_name":"prevention & control","is_major_topic":false},{"descriptor_ui":"D006801","descriptor_name":"Humans","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D007938","descriptor_name":"Leukemia","qualifier_ui":"Q000453","qualifier_name":"epidemiology","is_major_topic":false},{"descriptor_ui":"D008297","descriptor_name":"Male","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D011087","descriptor_name":"Polycythemia Vera","qualifier_ui":"Q000150","qualifier_name":"complications","is_major_topic":false},{"descriptor_ui":"D011087","descriptor_name":"Polycythemia Vera","qualifier_ui":"Q000453","qualifier_name":"epidemiology","is_major_topic":true},{"descriptor_ui":"D011087","descriptor_name":"Polycythemia Vera","qualifier_ui":"Q000628","qualifier_name":"therapy","is_major_topic":false},{"descriptor_ui":"D011230","descriptor_name":"Precancerous Conditions","qualifier_ui":"Q000453","qualifier_name":"epidemiology","is_major_topic":false},{"descriptor_ui":"D011247","descriptor_name":"Pregnancy","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D011250","descriptor_name":"Pregnancy Complications, Hematologic","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D011256","descriptor_name":"Pregnancy Outcome","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D011379","descriptor_name":"Prognosis","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D012306","descriptor_name":"Risk","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D013922","descriptor_name":"Thrombocytosis","qualifier_ui":"Q000150","qualifier_name":"complications","is_major_topic":false},{"descriptor_ui":"D013922","descriptor_name":"Thrombocytosis","qualifier_ui":"Q000188","qualifier_name":"drug therapy","is_major_topic":false},{"descriptor_ui":"D013922","descriptor_name":"Thrombocytosis","qualifier_ui":"Q000453","qualifier_name":"epidemiology","is_major_topic":true},{"descriptor_ui":"D013923","descriptor_name":"Thromboembolism","qualifier_ui":"Q000209","qualifier_name":"etiology","is_major_topic":false},{"descriptor_ui":"D013923","descriptor_name":"Thromboembolism","qualifier_ui":"Q000453","qualifier_name":"epidemiology","is_major_topic":false},{"descriptor_ui":"D013923","descriptor_name":"Thromboembolism","qualifier_ui":"Q000517","qualifier_name":"prevention & control","is_major_topic":false},{"descriptor_ui":"D015331","descriptor_name":"Cohort Studies","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D015994","descriptor_name":"Incidence","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D015996","descriptor_name":"Survival Rate","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D017668","descriptor_name":"Age of Onset","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false}],"locations_count":2,"locations":[{"id":"pmid:8039752","doi":null,"is_oa":false,"landing_page_url":"https://pubmed.ncbi.nlm.nih.gov/8039752","pdf_url":null,"source":{"id":"https://openalex.org/S4306525036","display_name":"PubMed","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I1299303238","host_organization_name":"National Institutes of Health","host_organization_lineage":["https://openalex.org/I1299303238"],"host_organization_lineage_names":[],"type":"repository"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Haematologica","raw_type":"Journal Article"},{"id":"mag:27612425","doi":null,"is_oa":false,"landing_page_url":"https://pubmed.ncbi.nlm.nih.gov/8039752","pdf_url":null,"source":{"id":"https://openalex.org/S4306525036","display_name":"PubMed","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I1299303238","host_organization_name":"National Institutes of Health","host_organization_lineage":["https://openalex.org/I1299303238"],"host_organization_lineage_names":[],"type":"repository"},"license":null,"license_id":null,"version":null,"is_accepted":false,"is_published":false,"raw_source_name":"PubMed","raw_type":null}],"best_oa_location":null,"sustainable_development_goals":[{"score":1.0,"display_name":"Good health and well-being","id":"https://metadata.un.org/sdg/3"}],"study_designs":[{"display_name":"Observational Study","id":"https://openalex.org/study-designs/observational-study"}],"awards":[],"funders":[],"has_content":{"pdf":false,"grobid_xml":false},"content_urls":null,"referenced_works_count":36,"referenced_works":["https://openalex.org/W1527347967","https://openalex.org/W1581466567","https://openalex.org/W1581665297","https://openalex.org/W1604315162","https://openalex.org/W1757407923","https://openalex.org/W1940241680","https://openalex.org/W1973980688","https://openalex.org/W1996866500","https://openalex.org/W1999528962","https://openalex.org/W2008828675","https://openalex.org/W2038608495","https://openalex.org/W2041440766","https://openalex.org/W2042797183","https://openalex.org/W2044563319","https://openalex.org/W2050658928","https://openalex.org/W2059600528","https://openalex.org/W2064054320","https://openalex.org/W2078874550","https://openalex.org/W2083457841","https://openalex.org/W2085005924","https://openalex.org/W2093297340","https://openalex.org/W2094390470","https://openalex.org/W2103441770","https://openalex.org/W2114751549","https://openalex.org/W2119180969","https://openalex.org/W2123549011","https://openalex.org/W2124295966","https://openalex.org/W2130894399","https://openalex.org/W2136101247","https://openalex.org/W2145606954","https://openalex.org/W2146501164","https://openalex.org/W2157734591","https://openalex.org/W2160609925","https://openalex.org/W2161596491","https://openalex.org/W2162771356","https://openalex.org/W2262414037"],"related_works":["https://openalex.org/W7123420068","https://openalex.org/W2911450427","https://openalex.org/W3006990862","https://openalex.org/W2949395018","https://openalex.org/W2952154478","https://openalex.org/W4403815952","https://openalex.org/W2320735351","https://openalex.org/W2979358798","https://openalex.org/W1982944047","https://openalex.org/W2788166434"],"abstract_inverted_index":{"BACKGROUND":[0],"AND":[1],"METHODS:":[2],"Polycythemia":[3],"vera":[4],"(PV)":[5],"and":[6,27,57,70,88,109,123,145,161,175,181,183,193,256,275,293],"essential":[7],"thrombocythemia":[8],"(ET)":[9],"in":[10,86,95,103,110,118,124,140,143,149,152,179,185,242,247,254,270],"young":[11,271,279,291],"patients":[12,26,46,63,105,112,121,127,159,164,197,214,222,238,272,292],"are":[13,268,301],"rarely":[14],"reported.":[15],"Their":[16],"natural":[17],"histories":[18],"seem":[19],"to":[20,50,304],"differ":[21],"from":[22],"those":[23],"of":[24,43,54,156,189,209,227,232,313],"older":[25],"the":[28,52,58,132,306],"best":[29,307],"treatment":[30,295,308],"is":[31],"still":[32],"uncertain.":[33],"In":[34,262],"this":[35,310],"follow-up":[36,78,117,147,177],"study":[37],"we":[38],"have":[39],"evaluated":[40],"a":[41,282],"cohort":[42],"64":[44],"consecutive":[45],"younger":[47],"than":[48],"40":[49],"determine":[51],"incidence":[53],"thrombohemorrhagic":[55,266],"events":[56,130,234],"long-term":[59],"outcome.":[60],"RESULTS:":[61],"Twenty-eight":[62],"(20":[64],"M;":[65],"8":[66],"F)":[67],"had":[68,166,198,215],"PV,":[69,141,150,180],"36":[71],"ET":[72,126,163,196,237,255,258],"(21":[73],"F,":[74],"15":[75],"M).":[76],"Mean":[77],"was":[79,207],"8.2":[80],"years":[81,90],"(range":[82,91],"4":[83],"months-16.7":[84],"years)":[85,94],"PV":[87,108,120,158,192,213,221,274],"6.5":[89],"5":[92],"months-15":[93],"ET.":[96,186],"Thrombohemorrhagic":[97],"symptoms":[98],"were":[99,131,173],"present":[100,269],"at":[101,137,171],"diagnosis":[102,138,172],"10/28":[104],"(35%)":[106],"with":[107,114,273],"12/36":[111],"(33%)":[113],"ET;":[115],"during":[116,146,176],"15/28":[119],"(53%)":[122],"13/36":[125],"(36%).":[128],"Thrombotic":[129],"most":[133],"frequent":[134],"symptoms,":[135],"both":[136],"(52%":[139],"65%":[142],"ET)":[144],"(43%":[148],"52%":[151],"ET).":[153],"A":[154,187],"total":[155,188],"19/28":[157],"(67%)":[160],"17/36":[162],"(47%)":[165],"thrombotic":[167,217,233,245],"complications.":[168],"Hemorrhagic":[169],"complications":[170,218,267],"4%":[174],"13%":[178,184],"15%":[182],"5/28":[190],"(18%)":[191],"6/36":[194],"(17%)":[195],"hemorrhagic":[199],"events.":[200,211],"No":[201,250],"laboratory":[202],"parameter,":[203],"including":[204],"platelet":[205],"count,":[206],"predictive":[208],"these":[210],"Five":[212],"major":[216,240],"(18%).":[219],"Four":[220,236],"died":[223],"(14%),":[224],"2":[225,230],"because":[226,231],"ANLL":[228],"(7%),":[229],"(7%).":[235],"experienced":[239],"complications,":[241],"three":[243],"cases":[244],"(8.3%),":[246],"one":[248],"hemorrhagic.":[249],"leukemic":[251],"transformation":[252],"occurred":[253],"no":[257],"patient":[259],"died.":[260],"CONCLUSIONS:":[261],"our":[263],"experience,":[264],"severe":[265],"ET,":[276],"which":[277],"excludes":[278],"age":[280],"as":[281],"favorable":[283],"prognostic":[284],"factor.":[285],"Treatment":[286],"also":[287],"seems":[288],"advisable":[289],"for":[290,309],"myelosuppressive":[294],"might":[296],"be":[297],"required.":[298],"Prospective":[299],"studies":[300],"urgently":[302],"needed":[303],"assess":[305],"particular":[311],"subset":[312],"patients.":[314]},"counts_by_year":[{"year":2017,"cited_by_count":2},{"year":2016,"cited_by_count":2},{"year":2013,"cited_by_count":1},{"year":2012,"cited_by_count":1}],"updated_date":"2026-11-04T10:54:05.112534","created_date":"2016-06-24T00:00:00"}