{"id":"https://openalex.org/W2232659909","doi":null,"title":"Paroxysmal nocturnal hemoglobinuria.","display_name":"Paroxysmal nocturnal hemoglobinuria.","publication_year":2004,"publication_date":"2004-01-01","ids":{"openalex":"https://openalex.org/W2232659909","mag":"2232659909","pmid":"https://pubmed.ncbi.nlm.nih.gov/15314892"},"language":"en","primary_location":{"id":"pmid:15314892","doi":null,"is_oa":false,"landing_page_url":"https://pubmed.ncbi.nlm.nih.gov/15314892","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":"Clinical laboratory science : journal of the American Society for Medical Technology","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/A5103827472","display_name":"Larry J. Smith","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I1334819555","display_name":"Memorial Sloan Kettering Cancer Center","ror":"https://ror.org/02yrq0923","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I1334819555"]}],"countries":["US"],"is_corresponding":true,"raw_author_name":"Larry J Smith","raw_affiliation_strings":["Department of Clinical Laboratories, Memorial Sloan-Kettering Cancer Center, 1275 York Avenue, New York, NY 10021, USA. Smith7@mskcc.org","Department of Clinical Laboratories, Memorial Sloan-Kettering Cancer Center, 1275 York Avenue, New York, NY 10021, USA"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Clinical Laboratories, Memorial Sloan-Kettering Cancer Center, 1275 York Avenue, New York, NY 10021, USA. Smith7@mskcc.org","institution_ids":["https://openalex.org/I1334819555"]},{"raw_affiliation_string":"Department of Clinical Laboratories, Memorial Sloan-Kettering Cancer Center, 1275 York Avenue, New York, NY 10021, USA","institution_ids":["https://openalex.org/I1334819555"]}]}],"institutions":[],"countries_distinct_count":1,"institutions_distinct_count":1,"corresponding_author_ids":["https://openalex.org/A5103827472"],"corresponding_institution_ids":["https://openalex.org/I1334819555"],"apc_list":null,"apc_paid":null,"fwci":1.2653,"has_fulltext":false,"cited_by_count":24,"citation_normalized_percentile":{"value":0.79179471,"is_in_top_1_percent":false,"is_in_top_10_percent":false},"cited_by_percentile_year":{"min":88,"max":95},"biblio":{"volume":"17","issue":"3","first_page":"172","last_page":"7"},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T10993","display_name":"Complement system in diseases","score":0.9940999746322632,"subfield":{"id":"https://openalex.org/subfields/2403","display_name":"Immunology"},"field":{"id":"https://openalex.org/fields/24","display_name":"Immunology and Microbiology"},"domain":{"id":"https://openalex.org/domains/1","display_name":"Life Sciences"}},"topics":[{"id":"https://openalex.org/T10993","display_name":"Complement system in diseases","score":0.9940999746322632,"subfield":{"id":"https://openalex.org/subfields/2403","display_name":"Immunology"},"field":{"id":"https://openalex.org/fields/24","display_name":"Immunology and Microbiology"},"domain":{"id":"https://openalex.org/domains/1","display_name":"Life Sciences"}},{"id":"https://openalex.org/T10554","display_name":"Hemoglobinopathies and Related Disorders","score":0.0010000000474974513,"subfield":{"id":"https://openalex.org/subfields/2716","display_name":"Genetics (clinical)"},"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/T11660","display_name":"Blood groups and transfusion","score":0.0007999999797903001,"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/paroxysmal-nocturnal-hemoglobinuria","display_name":"paroxysmal nocturnal hemoglobinuria","score":1.0},{"id":"https://openalex.org/keywords/intravascular-hemolysis","display_name":"intravascular hemolysis","score":0.8830000162124634},{"id":"https://openalex.org/keywords/somatic-mutations","display_name":"somatic mutations","score":0.6359999775886536},{"id":"https://openalex.org/keywords/hematopoietic-stem-cell-disorders","display_name":"hematopoietic stem cell disorders","score":0.8080000281333923},{"id":"https://openalex.org/keywords/glycosylphosphatidylinositol","display_name":"glycosylphosphatidylinositol","score":0.8640000224113464},{"id":"https://openalex.org/keywords/cd55","display_name":"CD55","score":0.9589999914169312},{"id":"https://openalex.org/keywords/cd59","display_name":"CD59","score":1.0},{"id":"https://openalex.org/keywords/complement-mediated-cell-lysis","display_name":"complement-mediated lysis","score":0.8059999942779541},{"id":"https://openalex.org/keywords/venous-thrombosis","display_name":"venous thrombosis","score":0.8500000238418579},{"id":"https://openalex.org/keywords/aplastic-anemia","display_name":"aplastic anemia","score":0.9179999828338623},{"id":"https://openalex.org/keywords/myelodysplastic-syndrome","display_name":"myelodysplastic syndrome","score":0.9980000257492065},{"id":"https://openalex.org/keywords/acute-leukemia","display_name":"acute leukemia","score":0.7609999775886536},{"id":"https://openalex.org/keywords/hematopoietic-stem-cell-transplantation","display_name":"hematopoietic stem cell transplantation","score":0.8939999938011169},{"id":"https://openalex.org/keywords/blood-transfusion-therapy","display_name":"transfusion therapy","score":0.8920000195503235},{"id":"https://openalex.org/keywords/anticoagulation","display_name":"anticoagulation","score":0.7990000247955322},{"id":"https://openalex.org/keywords/antibiotic-therapy","display_name":"antibiotic therapy","score":0.44600000977516174}],"concepts":[{"id":"https://openalex.org/C2777346216","wikidata":"https://www.wikidata.org/wiki/Q1479494","display_name":"Paroxysmal nocturnal hemoglobinuria","level":2,"score":0.9793078899383545},{"id":"https://openalex.org/C2781440808","wikidata":"https://www.wikidata.org/wiki/Q846316","display_name":"Aplastic anemia","level":3,"score":0.7097674012184143},{"id":"https://openalex.org/C71924100","wikidata":"https://www.wikidata.org/wiki/Q11190","display_name":"Medicine","level":0,"score":0.6086596846580505},{"id":"https://openalex.org/C2779902561","wikidata":"https://www.wikidata.org/wiki/Q471706","display_name":"Hemolysis","level":2,"score":0.5934956073760986},{"id":"https://openalex.org/C2778639011","wikidata":"https://www.wikidata.org/wiki/Q1553850","display_name":"Hemoglobinuria","level":3,"score":0.5617398619651794},{"id":"https://openalex.org/C2776175824","wikidata":"https://www.wikidata.org/wiki/Q1145668","display_name":"Hemolytic anemia","level":2,"score":0.5141308903694153},{"id":"https://openalex.org/C2780817109","wikidata":"https://www.wikidata.org/wiki/Q954625","display_name":"Myelodysplastic syndromes","level":3,"score":0.4835790991783142},{"id":"https://openalex.org/C2777991916","wikidata":"https://www.wikidata.org/wiki/Q421617","display_name":"Eculizumab","level":4,"score":0.481415718793869},{"id":"https://openalex.org/C203014093","wikidata":"https://www.wikidata.org/wiki/Q101929","display_name":"Immunology","level":1,"score":0.44435450434684753},{"id":"https://openalex.org/C2780525284","wikidata":"https://www.wikidata.org/wiki/Q7882181","display_name":"Bone marrow failure","level":4,"score":0.4420396685600281},{"id":"https://openalex.org/C2778248108","wikidata":"https://www.wikidata.org/wiki/Q5445","display_name":"Anemia","level":2,"score":0.43105554580688477},{"id":"https://openalex.org/C28328180","wikidata":"https://www.wikidata.org/wiki/Q48196","display_name":"Stem cell","level":2,"score":0.42597782611846924},{"id":"https://openalex.org/C2777408962","wikidata":"https://www.wikidata.org/wiki/Q1128050","display_name":"Hematopoietic stem cell transplantation","level":3,"score":0.4101768136024475},{"id":"https://openalex.org/C126322002","wikidata":"https://www.wikidata.org/wiki/Q11180","display_name":"Internal medicine","level":1,"score":0.3874293267726898},{"id":"https://openalex.org/C2780007613","wikidata":"https://www.wikidata.org/wiki/Q546523","display_name":"Bone marrow","level":2,"score":0.3401687443256378},{"id":"https://openalex.org/C90924648","wikidata":"https://www.wikidata.org/wiki/Q120569","display_name":"Gastroenterology","level":1,"score":0.33047014474868774},{"id":"https://openalex.org/C109159458","wikidata":"https://www.wikidata.org/wiki/Q919283","display_name":"Haematopoiesis","level":3,"score":0.29364240169525146},{"id":"https://openalex.org/C2911091166","wikidata":"https://www.wikidata.org/wiki/Q106419912","display_name":"Transplantation","level":2,"score":0.20581060647964478},{"id":"https://openalex.org/C86803240","wikidata":"https://www.wikidata.org/wiki/Q420","display_name":"Biology","level":0,"score":0.17802736163139343},{"id":"https://openalex.org/C111684460","wikidata":"https://www.wikidata.org/wiki/Q334848","display_name":"Complement system","level":3,"score":0.15811580419540405},{"id":"https://openalex.org/C8891405","wikidata":"https://www.wikidata.org/wiki/Q1059","display_name":"Immune system","level":2,"score":0.11266785860061646},{"id":"https://openalex.org/C54355233","wikidata":"https://www.wikidata.org/wiki/Q7162","display_name":"Genetics","level":1,"score":0.0}],"mesh":[{"descriptor_ui":"D000740","descriptor_name":"Anemia","qualifier_ui":"Q000209","qualifier_name":"etiology","is_major_topic":false},{"descriptor_ui":"D003937","descriptor_name":"Diagnosis, Differential","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D006457","descriptor_name":"Hemoglobinuria, Paroxysmal","qualifier_ui":"Q000175","qualifier_name":"diagnosis","is_major_topic":true},{"descriptor_ui":"D006457","descriptor_name":"Hemoglobinuria, Paroxysmal","qualifier_ui":"Q000503","qualifier_name":"physiopathology","is_major_topic":true},{"descriptor_ui":"D006457","descriptor_name":"Hemoglobinuria, Paroxysmal","qualifier_ui":"Q000628","qualifier_name":"therapy","is_major_topic":true},{"descriptor_ui":"D006801","descriptor_name":"Humans","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":"D012720","descriptor_name":"Severity of Illness Index","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D018380","descriptor_name":"Hematopoietic Stem Cell Transplantation","qualifier_ui":null,"qualifier_name":null,"is_major_topic":true}],"locations_count":2,"locations":[{"id":"pmid:15314892","doi":null,"is_oa":false,"landing_page_url":"https://pubmed.ncbi.nlm.nih.gov/15314892","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":"Clinical laboratory science : journal of the American Society for Medical Technology","raw_type":"Journal Article"},{"id":"mag:2232659909","doi":null,"is_oa":false,"landing_page_url":"https://pubmed.ncbi.nlm.nih.gov/15314892","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":[{"id":"https://metadata.un.org/sdg/3","display_name":"Good health and well-being","score":1.0}],"study_designs":[],"awards":[],"funders":[],"has_content":{"pdf":false,"grobid_xml":false},"content_urls":null,"referenced_works_count":0,"referenced_works":[],"related_works":["https://openalex.org/W1974475028","https://openalex.org/W2419250561","https://openalex.org/W1863721355","https://openalex.org/W4251511439","https://openalex.org/W3037780531","https://openalex.org/W7099191097","https://openalex.org/W2016113109","https://openalex.org/W2002702340","https://openalex.org/W7127038929","https://openalex.org/W2488534745"],"abstract_inverted_index":{"Paroxysmal":[0],"nocturnal":[1],"hemoglobinuria":[2],"(PNH)":[3],"is":[4,22,92,110],"an":[5],"acquired":[6],"clonal":[7],"stem":[8,19,122],"cell":[9,89,123],"disorder":[10],"resulting":[11],"from":[12,49],"a":[13,34],"somatic":[14],"mutation":[15],"in":[16,66],"the":[17,52,57,79],"hematopoietic":[18],"cell.":[20],"It":[21],"characterized":[23],"by":[24],"intravascular":[25],"hemolysis,":[26],"cytopenias,":[27],"frequent":[28],"infections,":[29],"bone":[30],"marrow":[31],"hypoplasia,":[32],"and":[33,61,74,87,99,102,118],"high":[35],"incidence":[36],"of":[37,114],"life-threatening":[38],"venous":[39],"thrombosis.":[40],"An":[41],"absent":[42],"glycosylphosphatidylinositol":[43],"(GPI)-anchored":[44],"receptor":[45],"prevents":[46],"several":[47],"proteins":[48],"binding":[50],"to":[51],"erythrocyte":[53],"membrane.":[54],"These":[55],"include":[56],"complement-regulatory":[58],"proteins,":[59],"CD55":[60],"CD59,":[62],"whose":[63],"absence":[64],"results":[65],"enhanced":[67],"complement-mediated":[68],"lysis.":[69],"Patients":[70],"present":[71],"with":[72],"anemia":[73],"hemoglobinuria.":[75],"Laboratory":[76],"diagnosis":[77],"includes":[78],"sucrose":[80],"hemolysis":[81,85],"test,":[82,86],"Ham":[83],"acid":[84],"fluorescent-activated":[88],"analysis.":[90],"There":[91],"considerable":[93],"overlap":[94],"between":[95],"PNH,":[96],"aplastic":[97],"anemia,":[98],"myelodysplastic":[100],"syndrome":[101],"some":[103],"cases":[104],"evolve":[105],"into":[106],"acute":[107],"leukemia.":[108],"Treatment":[109],"mainly":[111],"supportive":[112],"consisting":[113],"transfusion":[115],"therapy,":[116],"anticoagulation,":[117],"antibiotic":[119],"therapy.":[120],"Hematopoietic":[121],"transplantation":[124],"may":[125],"be":[126],"curative.":[127]},"counts_by_year":[{"year":2017,"cited_by_count":1},{"year":2016,"cited_by_count":1},{"year":2015,"cited_by_count":1},{"year":2014,"cited_by_count":1},{"year":2013,"cited_by_count":2},{"year":2012,"cited_by_count":1}],"updated_date":"2026-11-04T10:54:05.112534","created_date":"2016-06-24T00:00:00"}
