{"id":"https://openalex.org/W7203501537","doi":"https://doi.org/10.1097/cji.0000000000000614","title":"Immune Checkpoint Inhibitor-Associated Hemophagocytic Lymphohistiocytosis: Clinical Presentation and Outcomes in Three Patients","display_name":"Immune Checkpoint Inhibitor-Associated Hemophagocytic Lymphohistiocytosis: Clinical Presentation and Outcomes in Three Patients","publication_year":2026,"publication_date":"2026-08-14","ids":{"openalex":"https://openalex.org/W7203501537","doi":"https://doi.org/10.1097/cji.0000000000000614","pmid":"https://pubmed.ncbi.nlm.nih.gov/42598906"},"language":"en","primary_location":{"id":"doi:10.1097/cji.0000000000000614","doi":"https://doi.org/10.1097/cji.0000000000000614","is_oa":false,"landing_page_url":"https://doi.org/10.1097/cji.0000000000000614","pdf_url":null,"source":{"id":"https://openalex.org/S177451610","display_name":"Journal of Immunotherapy","issn_l":"1524-9557","issn":["1524-9557","1537-4513"],"is_oa":false,"is_in_doaj":false,"is_core":true,"listed_in":["cwts-core","doyens","jufo-1","ki-jl-1","medline","norway-1"],"host_organization":"https://openalex.org/P4310315671","host_organization_name":"Lippincott Williams & Wilkins","host_organization_lineage":["https://openalex.org/P4310315671","https://openalex.org/P4310318547"],"host_organization_lineage_names":["Lippincott Williams & Wilkins","Wolters Kluwer"],"type":"journal"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Journal of Immunotherapy","raw_type":"journal-article"},"volume":null,"type":"article","indexed_in":["crossref","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/A5147208946","display_name":"Mayte G. Martinez-Garza","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I98461037","display_name":"Tecnol\u00f3gico de Monterrey","ror":"https://ror.org/03ayjn504","country_code":"MX","type":"education","lineage":["https://openalex.org/I98461037"]}],"countries":["MX"],"is_corresponding":false,"raw_author_name":"Mayte G. Martinez-Garza","raw_affiliation_strings":["Tecnologico de Monterrey, School of Medicine and Health Sciences, Monterrey, Nuevo Leon, Mexico"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Tecnologico de Monterrey, School of Medicine and Health Sciences, Monterrey, Nuevo Leon, Mexico","institution_ids":["https://openalex.org/I98461037"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5013809001","display_name":"Effrosyni Apostolidou","orcid":"https://orcid.org/0000-0002-9337-3089","observed_orcids":["https://orcid.org/0000-0002-9337-3089"]},"institutions":[{"id":"https://openalex.org/I1343551460","display_name":"The University of Texas MD Anderson Cancer Center","ror":"https://ror.org/04twxam07","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I1343551460"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Effrosyni Apostolidou","raw_affiliation_strings":["Section of Benign Hematology, The University of Texas MD Anderson Cancer Center, Houston TX"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Section of Benign Hematology, The University of Texas MD Anderson Cancer Center, Houston TX","institution_ids":["https://openalex.org/I1343551460"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5000738451","display_name":"Hugo R. Lara-Martinez","orcid":"https://orcid.org/0000-0003-1087-6324","observed_orcids":["https://orcid.org/0000-0003-1087-6324"]},"institutions":[{"id":"https://openalex.org/I1343551460","display_name":"The University of Texas MD Anderson Cancer Center","ror":"https://ror.org/04twxam07","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I1343551460"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Hugo R. Lara-Martinez","raw_affiliation_strings":["Section of Benign Hematology, The University of Texas MD Anderson Cancer Center, Houston TX"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Section of Benign Hematology, The University of Texas MD Anderson Cancer Center, Houston TX","institution_ids":["https://openalex.org/I1343551460"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5038157897","display_name":"Thein Hlaing Oo","orcid":"https://orcid.org/0000-0002-5201-731X","observed_orcids":["https://orcid.org/0000-0002-5201-731X"]},"institutions":[{"id":"https://openalex.org/I1343551460","display_name":"The University of Texas MD Anderson Cancer Center","ror":"https://ror.org/04twxam07","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I1343551460"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Thein H. Oo","raw_affiliation_strings":["Section of Benign Hematology, The University of Texas MD Anderson Cancer Center, Houston TX"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Section of Benign Hematology, The University of Texas MD Anderson Cancer Center, Houston TX","institution_ids":["https://openalex.org/I1343551460"]}]},{"author_position":"last","author":{"id":"https://openalex.org/A5046413977","display_name":"Cristhiam Mauricio Rojas Hernandez","orcid":"https://orcid.org/0000-0003-3890-3929","observed_orcids":["https://orcid.org/0000-0003-3890-3929"]},"institutions":[{"id":"https://openalex.org/I1343551460","display_name":"The University of Texas MD Anderson Cancer Center","ror":"https://ror.org/04twxam07","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I1343551460"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Cristhiam M. Rojas-Hernandez","raw_affiliation_strings":["Section of Benign Hematology, The University of Texas MD Anderson Cancer Center, Houston TX"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Section of Benign Hematology, The University of Texas MD Anderson Cancer Center, Houston TX","institution_ids":["https://openalex.org/I1343551460"]}]}],"institutions":[],"countries_distinct_count":2,"institutions_distinct_count":2,"corresponding_author_ids":[],"corresponding_institution_ids":[],"apc_list":null,"apc_paid":null,"fwci":4.6525,"has_fulltext":false,"cited_by_count":1,"citation_normalized_percentile":{"value":0.95740963,"is_in_top_1_percent":false,"is_in_top_10_percent":true},"cited_by_percentile_year":{"min":93,"max":96},"biblio":{"volume":"49","issue":"8","first_page":"302","last_page":"306"},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T11616","display_name":"Autoimmune and Inflammatory Disorders Research","score":0.9984999895095825,"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":0.9984999895095825,"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/T10158","display_name":"Cancer Immunotherapy and Biomarkers","score":0.000699999975040555,"subfield":{"id":"https://openalex.org/subfields/2730","display_name":"Oncology"},"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/T12845","display_name":"Methemoglobinemia and Tumor Lysis Syndrome","score":9.999999747378752e-05,"subfield":{"id":"https://openalex.org/subfields/2740","display_name":"Pulmonary and Respiratory Medicine"},"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":0.9639999866485596},{"id":"https://openalex.org/keywords/immune-checkpoint-inhibitors","display_name":"immune checkpoint inhibitors","score":0.9660000205039978},{"id":"https://openalex.org/keywords/hyperinflammatory-syndrome","display_name":"hyperinflammatory syndrome","score":0.9100000262260437},{"id":"https://openalex.org/keywords/systemic-inflammation","display_name":"systemic inflammation","score":0.734000027179718},{"id":"https://openalex.org/keywords/dexamethasone","display_name":"dexamethasone","score":0.9440000057220459},{"id":"https://openalex.org/keywords/anakinra","display_name":"anakinra","score":0.9929999709129333},{"id":"https://openalex.org/keywords/ruxolitinib","display_name":"ruxolitinib","score":1.0},{"id":"https://openalex.org/keywords/tocilizumab","display_name":"tocilizumab","score":1.0},{"id":"https://openalex.org/keywords/immunosuppressive-therapy","display_name":"immunosuppressive therapy","score":0.9319999814033508},{"id":"https://openalex.org/keywords/misdiagnosis","display_name":"misdiagnosis","score":0.6349999904632568}],"concepts":[{"id":"https://openalex.org/C71924100","wikidata":"https://www.wikidata.org/wiki/Q11190","display_name":"Medicine","level":0,"score":0.8748999834060669},{"id":"https://openalex.org/C2779079688","wikidata":"https://www.wikidata.org/wiki/Q1642170","display_name":"Hemophagocytic lymphohistiocytosis","level":3,"score":0.6948999762535095},{"id":"https://openalex.org/C2780851360","wikidata":"https://www.wikidata.org/wiki/Q21686041","display_name":"Immune checkpoint","level":4,"score":0.5357000231742859},{"id":"https://openalex.org/C2780030458","wikidata":"https://www.wikidata.org/wiki/Q7041828","display_name":"Nivolumab","level":4,"score":0.5315999984741211},{"id":"https://openalex.org/C2780401358","wikidata":"https://www.wikidata.org/wiki/Q422252","display_name":"Dexamethasone","level":2,"score":0.47189998626708984},{"id":"https://openalex.org/C8891405","wikidata":"https://www.wikidata.org/wiki/Q1059","display_name":"Immune system","level":2,"score":0.4609000086784363},{"id":"https://openalex.org/C2781433595","wikidata":"https://www.wikidata.org/wiki/Q2459042","display_name":"Ipilimumab","level":4,"score":0.4406000077724457},{"id":"https://openalex.org/C203014093","wikidata":"https://www.wikidata.org/wiki/Q101929","display_name":"Immunology","level":1,"score":0.4185999929904938},{"id":"https://openalex.org/C2777601897","wikidata":"https://www.wikidata.org/wiki/Q3409113","display_name":"Presentation (obstetrics)","level":2,"score":0.4000999927520752},{"id":"https://openalex.org/C2777701055","wikidata":"https://www.wikidata.org/wiki/Q1427096","display_name":"Immunotherapy","level":3,"score":0.38589999079704285},{"id":"https://openalex.org/C143998085","wikidata":"https://www.wikidata.org/wiki/Q162555","display_name":"Oncology","level":1,"score":0.3452000021934509},{"id":"https://openalex.org/C2778426790","wikidata":"https://www.wikidata.org/wiki/Q1649580","display_name":"Organ dysfunction","level":3,"score":0.3206999897956848},{"id":"https://openalex.org/C126322002","wikidata":"https://www.wikidata.org/wiki/Q11180","display_name":"Internal medicine","level":1,"score":0.3158999979496002},{"id":"https://openalex.org/C21705690","wikidata":"https://www.wikidata.org/wiki/Q2907609","display_name":"CTLA-4","level":4,"score":0.30730000138282776},{"id":"https://openalex.org/C2776914184","wikidata":"https://www.wikidata.org/wiki/Q101991","display_name":"Inflammation","level":2,"score":0.3043999969959259},{"id":"https://openalex.org/C2781090800","wikidata":"https://www.wikidata.org/wiki/Q776030","display_name":"Systemic inflammatory response syndrome","level":3,"score":0.2806999981403351},{"id":"https://openalex.org/C2779778235","wikidata":"https://www.wikidata.org/wiki/Q539986","display_name":"Immune reconstitution inflammatory syndrome","level":5,"score":0.26820001006126404},{"id":"https://openalex.org/C2781053074","wikidata":"https://www.wikidata.org/wiki/Q21100639","display_name":"PD-L1","level":4,"score":0.2565000057220459}],"mesh":[{"descriptor_ui":"D000082082","descriptor_name":"Immune Checkpoint Inhibitors","qualifier_ui":"Q000009","qualifier_name":"adverse effects","is_major_topic":true},{"descriptor_ui":"D000082082","descriptor_name":"Immune Checkpoint Inhibitors","qualifier_ui":"Q000009","qualifier_name":"adverse effects","is_major_topic":true},{"descriptor_ui":"D000082082","descriptor_name":"Immune Checkpoint Inhibitors","qualifier_ui":"Q000627","qualifier_name":"therapeutic use","is_major_topic":true},{"descriptor_ui":"D000082082","descriptor_name":"Immune Checkpoint Inhibitors","qualifier_ui":"Q000627","qualifier_name":"therapeutic use","is_major_topic":true},{"descriptor_ui":"D000368","descriptor_name":"Aged","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D000368","descriptor_name":"Aged","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D003907","descriptor_name":"Dexamethasone","qualifier_ui":"Q000627","qualifier_name":"therapeutic use","is_major_topic":false},{"descriptor_ui":"D003907","descriptor_name":"Dexamethasone","qualifier_ui":"Q000627","qualifier_name":"therapeutic use","is_major_topic":false},{"descriptor_ui":"D005260","descriptor_name":"Female","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":"D006801","descriptor_name":"Humans","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":"D007166","descriptor_name":"Immunosuppressive Agents","qualifier_ui":"Q000627","qualifier_name":"therapeutic use","is_major_topic":false},{"descriptor_ui":"D007166","descriptor_name":"Immunosuppressive Agents","qualifier_ui":"Q000627","qualifier_name":"therapeutic use","is_major_topic":false},{"descriptor_ui":"D008297","descriptor_name":"Male","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D008297","descriptor_name":"Male","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D008875","descriptor_name":"Middle Aged","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D016896","descriptor_name":"Treatment Outcome","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D016896","descriptor_name":"Treatment Outcome","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D051359","descriptor_name":"Lymphohistiocytosis, Hemophagocytic","qualifier_ui":"Q000175","qualifier_name":"diagnosis","is_major_topic":true},{"descriptor_ui":"D051359","descriptor_name":"Lymphohistiocytosis, Hemophagocytic","qualifier_ui":"Q000175","qualifier_name":"diagnosis","is_major_topic":true},{"descriptor_ui":"D051359","descriptor_name":"Lymphohistiocytosis, Hemophagocytic","qualifier_ui":"Q000188","qualifier_name":"drug therapy","is_major_topic":true},{"descriptor_ui":"D051359","descriptor_name":"Lymphohistiocytosis, Hemophagocytic","qualifier_ui":"Q000188","qualifier_name":"drug therapy","is_major_topic":true},{"descriptor_ui":"D051359","descriptor_name":"Lymphohistiocytosis, Hemophagocytic","qualifier_ui":"Q000209","qualifier_name":"etiology","is_major_topic":true},{"descriptor_ui":"D051359","descriptor_name":"Lymphohistiocytosis, Hemophagocytic","qualifier_ui":"Q000209","qualifier_name":"etiology","is_major_topic":true},{"descriptor_ui":"D061067","descriptor_name":"Antibodies, Monoclonal, Humanized","qualifier_ui":"Q000627","qualifier_name":"therapeutic use","is_major_topic":false},{"descriptor_ui":"D061067","descriptor_name":"Antibodies, Monoclonal, Humanized","qualifier_ui":"Q000627","qualifier_name":"therapeutic use","is_major_topic":false}],"locations_count":2,"locations":[{"id":"doi:10.1097/cji.0000000000000614","doi":"https://doi.org/10.1097/cji.0000000000000614","is_oa":false,"landing_page_url":"https://doi.org/10.1097/cji.0000000000000614","pdf_url":null,"source":{"id":"https://openalex.org/S177451610","display_name":"Journal of Immunotherapy","issn_l":"1524-9557","issn":["1524-9557","1537-4513"],"is_oa":false,"is_in_doaj":false,"is_core":true,"listed_in":["cwts-core","doyens","jufo-1","ki-jl-1","medline","norway-1"],"host_organization":"https://openalex.org/P4310315671","host_organization_name":"Lippincott Williams & Wilkins","host_organization_lineage":["https://openalex.org/P4310315671","https://openalex.org/P4310318547"],"host_organization_lineage_names":["Lippincott Williams & Wilkins","Wolters Kluwer"],"type":"journal"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Journal of Immunotherapy","raw_type":"journal-article"},{"id":"pmid:42598906","doi":null,"is_oa":false,"landing_page_url":"https://pubmed.ncbi.nlm.nih.gov/42598906","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":"Journal of immunotherapy (Hagerstown, Md. : 1997)","raw_type":"Journal Article"}],"best_oa_location":null,"sustainable_development_goals":[{"score":1.0,"display_name":"Good health and well-being","id":"https://metadata.un.org/sdg/3"}],"sustainable_development_goals_aurora":[{"score":0.7600465416908264,"display_name":"Good health and well-being","id":"https://metadata.un.org/sdg/3"}],"study_designs":[{"display_name":"Case Report","id":"https://openalex.org/study-designs/case-report"}],"awards":[],"funders":[],"has_content":{"grobid_xml":false,"pdf":false},"content_urls":null,"referenced_works_count":8,"referenced_works":["https://openalex.org/W2936019126","https://openalex.org/W2947217536","https://openalex.org/W3009455860","https://openalex.org/W3135040069","https://openalex.org/W4322769976","https://openalex.org/W4402987666","https://openalex.org/W4407162945","https://openalex.org/W7108664974"],"related_works":[],"abstract_inverted_index":{"Immune":[0],"checkpoint":[1,64,112],"inhibitor":[2,113],"therapy":[3],"has":[4],"become":[5],"increasingly":[6],"recognized":[7],"as":[8,78],"a":[9,14,96],"trigger":[10],"for":[11,101],"hemophagocytic":[12,66,102],"lymphohistiocytosis,":[13],"life-threatening":[15],"hyperinflammatory":[16],"syndrome":[17],"that":[18],"may":[19],"lead":[20],"to":[21],"organ":[22],"failure":[23],"without":[24],"timely":[25],"interventions.":[26],"Given":[27],"the":[28,43,92],"rarity":[29],"of":[30,70,94,99],"this":[31],"entity,":[32],"patients":[33,49,105],"are":[34],"often":[35],"misdiagnosed":[36],"with":[37,50,62,73,83,107],"more":[38],"common":[39],"oncologic":[40],"complications.":[41],"In":[42],"present":[44],"series,":[45],"we":[46],"describe":[47],"3":[48],"diverse":[51],"clinical":[52,85],"pictures":[53],"evaluated":[54],"at":[55],"hospital":[56],"admissions,":[57],"who":[58],"were":[59],"ultimately":[60],"diagnosed":[61],"immune":[63,111],"inhibitor-associated":[65],"lymphohistiocytosis.":[67],"Treatment":[68],"consisted":[69],"dexamethasone":[71],"combined":[72],"an":[74],"immunosuppressive":[75],"agent":[76],"such":[77],"anakinra,":[79],"ruxolitinib,":[80],"or":[81],"tocilizumab,":[82],"variable":[84],"responses":[86],"among":[87],"patients.":[88],"These":[89],"cases":[90],"highlight":[91],"importance":[93],"maintaining":[95],"high":[97],"index":[98],"suspicion":[100],"lymphohistiocytosis":[103],"in":[104],"presenting":[106],"systemic":[108],"inflammation":[109],"during":[110],"therapy.":[114]},"counts_by_year":[{"year":2026,"cited_by_count":1}],"updated_date":"2026-10-03T13:16:28.481748","created_date":"2026-08-15T00:00:00"}