{"id":"https://openalex.org/W204540028","doi":null,"title":"Advances in the management of hemophagocytic lymphohistiocytosis.","display_name":"Advances in the management of hemophagocytic lymphohistiocytosis.","publication_year":2000,"publication_date":"2000-07-01","ids":{"openalex":"https://openalex.org/W204540028","mag":"204540028","pmid":"https://pubmed.ncbi.nlm.nih.gov/10979202"},"language":"en","primary_location":{"id":"pmid:10979202","doi":null,"is_oa":false,"landing_page_url":"https://pubmed.ncbi.nlm.nih.gov/10979202","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":"International journal of hematology","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/A5086967218","display_name":"Shinsaku Imashuku","orcid":"https://orcid.org/0000-0001-9795-0819","observed_orcids":["https://orcid.org/0000-0001-9795-0819","https://orcid.org/0009-0001-0524-9898"]},"institutions":[{"id":"https://openalex.org/I4405255084","display_name":"Kyoto City Institute of Health and Environmental Sciences","ror":"https://ror.org/02ksnjk05","country_code":"JP","type":"facility","lineage":["https://openalex.org/I4405255084"]}],"countries":["JP"],"is_corresponding":true,"raw_author_name":"S Imashuku","raw_affiliation_strings":["Kyoto City Institute of Health and Environmental Sciences, Japan. shinim95@mbox.kyoto-inet.or.jp"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Kyoto City Institute of Health and Environmental Sciences, Japan. shinim95@mbox.kyoto-inet.or.jp","institution_ids":["https://openalex.org/I4405255084"]}]}],"institutions":[],"countries_distinct_count":1,"institutions_distinct_count":1,"corresponding_author_ids":["https://openalex.org/A5086967218"],"corresponding_institution_ids":["https://openalex.org/I4405255084"],"apc_list":null,"apc_paid":null,"fwci":4.0329,"has_fulltext":false,"cited_by_count":112,"citation_normalized_percentile":{"value":0.94077582,"is_in_top_1_percent":false,"is_in_top_10_percent":true},"cited_by_percentile_year":{"min":88,"max":99},"biblio":{"volume":"72","issue":"1","first_page":"1","last_page":"11"},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T11616","display_name":"Autoimmune and Inflammatory Disorders Research","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/T11616","display_name":"Autoimmune and Inflammatory Disorders Research","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/T11725","display_name":"Immunodeficiency and Autoimmune Disorders","score":0.0,"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/T11892","display_name":"Kawasaki Disease and Coronary Complications","score":0.0,"subfield":{"id":"https://openalex.org/subfields/2735","display_name":"Pediatrics, Perinatology and Child Health"},"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/hemophagocytic-lymphohistiocytosis","display_name":"hemophagocytic lymphohistiocytosis","score":0.9990000128746033},{"id":"https://openalex.org/keywords/familial-hemophagocytic-lymphohistiocytosis","display_name":"familial hemophagocytic lymphohistiocytosis","score":0.9890000224113464},{"id":"https://openalex.org/keywords/etoposide","display_name":"etoposide","score":0.6830000281333923},{"id":"https://openalex.org/keywords/corticosteroids","display_name":"corticosteroids","score":0.9829999804496765},{"id":"https://openalex.org/keywords/cyclosporine","display_name":"cyclosporine","score":0.9599999785423279},{"id":"https://openalex.org/keywords/stem-cell-transplantation","display_name":"stem cell transplantation","score":0.8989999890327454},{"id":"https://openalex.org/keywords/immunotherapy","display_name":"immunotherapy","score":0.5690000057220459},{"id":"https://openalex.org/keywords/herpes-simplex-virus","display_name":"herpes simplex virus","score":0.6200000047683716},{"id":"https://openalex.org/keywords/adenovirus-type-7","display_name":"adenovirus type 7","score":0.8899999856948853},{"id":"https://openalex.org/keywords/cytokine-dysregulation","display_name":"cytokine imbalance","score":0.7009999752044678},{"id":"https://openalex.org/keywords/central-nervous-system-involvement","display_name":"central nervous system involvement","score":0.718999981880188},{"id":"https://openalex.org/keywords/early-diagnosis","display_name":"early diagnosis","score":0.5320000052452087},{"id":"https://openalex.org/keywords/pediatric-patients","display_name":"pediatric patients","score":0.4000000059604645}],"concepts":[{"id":"https://openalex.org/C2779079688","wikidata":"https://www.wikidata.org/wiki/Q1642170","display_name":"Hemophagocytic lymphohistiocytosis","level":3,"score":0.9534134864807129},{"id":"https://openalex.org/C71924100","wikidata":"https://www.wikidata.org/wiki/Q11190","display_name":"Medicine","level":0,"score":0.7335258722305298},{"id":"https://openalex.org/C2778119113","wikidata":"https://www.wikidata.org/wiki/Q418817","display_name":"Etoposide","level":3,"score":0.644314706325531},{"id":"https://openalex.org/C203014093","wikidata":"https://www.wikidata.org/wiki/Q101929","display_name":"Immunology","level":1,"score":0.5917428135871887},{"id":"https://openalex.org/C2779338263","wikidata":"https://www.wikidata.org/wiki/Q208414","display_name":"Lymphoma","level":2,"score":0.5440077781677246},{"id":"https://openalex.org/C2779134260","wikidata":"https://www.wikidata.org/wiki/Q12136","display_name":"Disease","level":2,"score":0.5357906222343445},{"id":"https://openalex.org/C8891405","wikidata":"https://www.wikidata.org/wiki/Q1059","display_name":"Immune system","level":2,"score":0.42950135469436646},{"id":"https://openalex.org/C187212893","wikidata":"https://www.wikidata.org/wiki/Q123028","display_name":"Pediatrics","level":1,"score":0.3288560211658478},{"id":"https://openalex.org/C2776694085","wikidata":"https://www.wikidata.org/wiki/Q974135","display_name":"Chemotherapy","level":2,"score":0.2782420814037323},{"id":"https://openalex.org/C126322002","wikidata":"https://www.wikidata.org/wiki/Q11180","display_name":"Internal medicine","level":1,"score":0.23253780603408813}],"mesh":[{"descriptor_ui":"D003937","descriptor_name":"Diagnosis, Differential","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D006801","descriptor_name":"Humans","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D012307","descriptor_name":"Risk Factors","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D015616","descriptor_name":"Histiocytosis, Non-Langerhans-Cell","qualifier_ui":"Q000145","qualifier_name":"classification","is_major_topic":false},{"descriptor_ui":"D015616","descriptor_name":"Histiocytosis, Non-Langerhans-Cell","qualifier_ui":"Q000175","qualifier_name":"diagnosis","is_major_topic":true},{"descriptor_ui":"D015616","descriptor_name":"Histiocytosis, Non-Langerhans-Cell","qualifier_ui":"Q000628","qualifier_name":"therapy","is_major_topic":true},{"descriptor_ui":"D019468","descriptor_name":"Disease Management","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false}],"locations_count":2,"locations":[{"id":"pmid:10979202","doi":null,"is_oa":false,"landing_page_url":"https://pubmed.ncbi.nlm.nih.gov/10979202","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":"International journal of hematology","raw_type":"Journal Article"},{"id":"mag:204540028","doi":null,"is_oa":false,"landing_page_url":"https://pubmed.ncbi.nlm.nih.gov/10979202","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":[{"display_name":"Good health and well-being","score":1.0,"id":"https://metadata.un.org/sdg/3"}],"study_designs":[],"awards":[],"funders":[],"has_content":{"grobid_xml":false,"pdf":false},"content_urls":null,"referenced_works_count":114,"referenced_works":["https://openalex.org/W54255155","https://openalex.org/W89839533","https://openalex.org/W99213633","https://openalex.org/W104330063","https://openalex.org/W145217207","https://openalex.org/W147559783","https://openalex.org/W158096899","https://openalex.org/W170573079","https://openalex.org/W178738040","https://openalex.org/W187791956","https://openalex.org/W197098624","https://openalex.org/W210433600","https://openalex.org/W624784516","https://openalex.org/W1487843912","https://openalex.org/W1497727675","https://openalex.org/W1510178802","https://openalex.org/W1848803438","https://openalex.org/W1875574217","https://openalex.org/W1909467642","https://openalex.org/W1924440436","https://openalex.org/W1925683597","https://openalex.org/W1969150464","https://openalex.org/W1970592114","https://openalex.org/W1972949749","https://openalex.org/W1974883812","https://openalex.org/W1976719341","https://openalex.org/W1980003220","https://openalex.org/W1980762484","https://openalex.org/W1982656985","https://openalex.org/W1986658169","https://openalex.org/W1991578501","https://openalex.org/W1992246456","https://openalex.org/W1992523064","https://openalex.org/W1993958476","https://openalex.org/W1998794304","https://openalex.org/W1999342420","https://openalex.org/W2000809021","https://openalex.org/W2002165051","https://openalex.org/W2004453731","https://openalex.org/W2007460330","https://openalex.org/W2008528405","https://openalex.org/W2008726620","https://openalex.org/W2009583354","https://openalex.org/W2009734601","https://openalex.org/W2015925408","https://openalex.org/W2016130839","https://openalex.org/W2020196713","https://openalex.org/W2021504799","https://openalex.org/W2023530038","https://openalex.org/W2030506725","https://openalex.org/W2036455688","https://openalex.org/W2037125126","https://openalex.org/W2037251946","https://openalex.org/W2037469410","https://openalex.org/W2038771281","https://openalex.org/W2039852679","https://openalex.org/W2041763575","https://openalex.org/W2046454420","https://openalex.org/W2047823796","https://openalex.org/W2048520028","https://openalex.org/W2051602856","https://openalex.org/W2058931989","https://openalex.org/W2061546795","https://openalex.org/W2062603648","https://openalex.org/W2064567786","https://openalex.org/W2066317932","https://openalex.org/W2069448906","https://openalex.org/W2074174279","https://openalex.org/W2081951127","https://openalex.org/W2082378607","https://openalex.org/W2089736326","https://openalex.org/W2089905151","https://openalex.org/W2089935211","https://openalex.org/W2092892553","https://openalex.org/W2095948475","https://openalex.org/W2099390515","https://openalex.org/W2102535678","https://openalex.org/W2106958355","https://openalex.org/W2117010463","https://openalex.org/W2119127315","https://openalex.org/W2125696453","https://openalex.org/W2131925046","https://openalex.org/W2136080892","https://openalex.org/W2140794890","https://openalex.org/W2144510654","https://openalex.org/W2146680365","https://openalex.org/W2149179920","https://openalex.org/W2153170729","https://openalex.org/W2153728910","https://openalex.org/W2156479425","https://openalex.org/W2162777258","https://openalex.org/W2164863699","https://openalex.org/W2168801583","https://openalex.org/W2171190856","https://openalex.org/W2173174278","https://openalex.org/W2261200687","https://openalex.org/W2318199891","https://openalex.org/W2321108727","https://openalex.org/W2321222078","https://openalex.org/W2330122844","https://openalex.org/W2331405793","https://openalex.org/W2333694726","https://openalex.org/W2335182598","https://openalex.org/W2341023780","https://openalex.org/W2394895925","https://openalex.org/W2401351612","https://openalex.org/W2412442891","https://openalex.org/W2413247543","https://openalex.org/W2426125013","https://openalex.org/W2461190456","https://openalex.org/W2464567171","https://openalex.org/W2469738557","https://openalex.org/W2886647778","https://openalex.org/W4285719527"],"related_works":["https://openalex.org/W2150680483","https://openalex.org/W2094052254","https://openalex.org/W7127040555","https://openalex.org/W2356126226","https://openalex.org/W2186785639","https://openalex.org/W2166811345","https://openalex.org/W4392304386","https://openalex.org/W2010286155","https://openalex.org/W2162251899","https://openalex.org/W2276557969"],"abstract_inverted_index":{"Hemophagocytic":[0],"lymphohistiocytosis":[1],"(HLH)":[2],"is":[3,88,200,220,252,268],"a":[4,31,41,107,145,248],"prototype":[5],"of":[6,33,43,51,84,104,110,117,129,147,186,201,225,236,244],"the":[7,81,130,222,233,237],"hemophagocytic":[8],"syndrome":[9],"and":[10,65,112,124,149,192],"occurs":[11,29],"most":[12,212],"often":[13],"in":[14,17,73,180,196,260,272],"children.":[15],"Progress":[16],"cytokine":[18],"research":[19],"has":[20],"now":[21,77],"made":[22],"it":[23,199],"possible":[24],"to":[25,204,215,231,242,256],"show":[26],"that":[27,79,170,221,264],"HLH":[28,52,58,63,72,195,218,226,245],"as":[30,169],"consequence":[32],"uncontrolled,":[34],"dysregulated":[35],"cellular":[36],"immune":[37],"reactivity":[38],"caused":[39],"by":[40,122,133],"number":[42],"different":[44],"underlying":[45,70,223],"diseases.":[46],"Three":[47],"major":[48],"risk":[49],"groups":[50],"can":[53,274],"be":[54,142,156,178,228,275],"identified:":[55],"(1)":[56],"familial":[57],"(FHL),":[59],"(2)":[60],"Epstein-Barr":[61],"virus-associated":[62],"(EBV-HLH),":[64],"(3)":[66],"life-threatening":[67],"infection-associated":[68],"or":[69,152,208],"disease-unknown":[71],"infancy.":[74],"Diagnostic":[75],"criteria":[76],"exist":[78],"allow":[80],"differential":[82],"diagnosis":[83],"these":[85,181],"groups,":[86],"which":[87,267],"important":[89,213],"because":[90],"distinct":[91],"therapeutic":[92],"measures":[93],"are":[94],"advised":[95],"for":[96,172,254,270],"each":[97],"group.":[98],"FHL":[99],"patients":[100],"require":[101],"immediate":[102],"application":[103,235],"immunochemotherapy":[105],"with":[106,115,144,158,164,258],"core":[108],"combination":[109,146],"corticosteroids":[111,148],"etoposide":[113],"together":[114],"monitoring":[116],"central":[118],"nervous":[119],"system":[120],"disease":[121],"early":[123,197,265],"repeated":[125],"magnetic":[126],"resonance":[127],"imaging":[128],"brain,":[131],"followed":[132],"timely":[134],"stem":[135],"cell":[136],"transplantation":[137],"(SCT).":[138],"EBV-HLH":[139],"should":[140,155],"also":[141,177],"treated":[143,157],"etoposide.":[150],"Aggressive":[151],"relapsed":[153],"cases":[154,185,246],"cyclosporin":[159],"A":[160],"and,":[161],"if":[162],"necessary,":[163],"more":[165],"intensive":[166],"chemotherapy,":[167],"such":[168],"used":[171],"non-Hodgkin's":[173],"lymphoma.":[174],"SCT":[175],"may":[176],"needed":[179],"refractory":[182],"cases.":[183],"In":[184],"herpes":[187],"simplex":[188],"virus,":[189],"adenovirus":[190],"7,":[191],"other":[193,261],"pathogen-undetermined":[194],"infancy,":[198],"great":[202],"importance":[203],"administer":[205],"appropriate":[206,238],"antiviral":[207],"antibacterial":[209],"agents.":[210],"The":[211],"point":[214],"make":[216],"regarding":[217],"treatment":[219],"cause":[224],"must":[227],"promptly":[229],"established":[230],"enable":[232],"rapid":[234],"therapy.":[239],"Currently,":[240],"30%":[241],"40%":[243],"have":[247],"poor":[249],"outcome.":[250],"It":[251],"necessary":[253],"hematologists":[255],"cooperate":[257],"specialists":[259],"fields":[262],"so":[263],"diagnosis,":[266],"critical":[269],"improvements":[271],"outcome,":[273],"made.":[276]},"counts_by_year":[{"year":2026,"cited_by_count":1},{"year":2023,"cited_by_count":2},{"year":2022,"cited_by_count":2},{"year":2021,"cited_by_count":2},{"year":2020,"cited_by_count":1},{"year":2019,"cited_by_count":4},{"year":2018,"cited_by_count":3},{"year":2017,"cited_by_count":2},{"year":2016,"cited_by_count":2},{"year":2015,"cited_by_count":3},{"year":2014,"cited_by_count":5},{"year":2013,"cited_by_count":6},{"year":2012,"cited_by_count":8}],"updated_date":"2026-11-07T11:25:07.637180","created_date":"2016-06-24T00:00:00"}