{"id":"https://openalex.org/W7225340303","doi":"https://doi.org/10.1007/s00296-026-06329-4","title":"Sirolimus-induced improvement of immune dysregulation associated hepatosplenomegaly in common variable immunodeficiency: a case-based review","display_name":"Sirolimus-induced improvement of immune dysregulation associated hepatosplenomegaly in common variable immunodeficiency: a case-based review","publication_year":2026,"publication_date":"2026-11-05","ids":{"openalex":"https://openalex.org/W7225340303","doi":"https://doi.org/10.1007/s00296-026-06329-4","pmid":"https://pubmed.ncbi.nlm.nih.gov/42986447","pmcid":"https://www.ncbi.nlm.nih.gov/pmc/articles/13740097"},"language":"en","primary_location":{"id":"doi:10.1007/s00296-026-06329-4","doi":"https://doi.org/10.1007/s00296-026-06329-4","is_oa":true,"landing_page_url":"https://doi.org/10.1007/s00296-026-06329-4","pdf_url":"https://link.springer.com/content/pdf/10.1007/s00296-026-06329-4.pdf","source":{"id":"https://openalex.org/S50823678","display_name":"Rheumatology International","issn_l":"0172-8172","issn":["0172-8172","1437-160X"],"is_oa":false,"is_in_doaj":false,"is_core":true,"listed_in":["anvur-scientific","cwts-core","dongbi-d","doyens","jufo-1","ki-jl-1","medline","norway-1","poland-70","russia-white-list-2","vabb-shw"],"host_organization":"https://openalex.org/P4310319900","host_organization_name":"Springer Science+Business Media","host_organization_lineage":["https://openalex.org/P4310319900","https://openalex.org/P4310319965"],"host_organization_lineage_names":["Springer Science+Business Media","Springer Nature"],"type":"journal"},"license":"cc-by-nc-nd","license_id":"https://openalex.org/licenses/cc-by-nc-nd","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Rheumatology International","raw_type":"journal-article"},"volume":null,"type":"article","is_traditional":true,"indexed_in":["crossref","pubmed"],"open_access":{"is_oa":true,"oa_status":"hybrid","oa_url":"https://link.springer.com/content/pdf/10.1007/s00296-026-06329-4.pdf","any_repository_has_fulltext":true},"authorships":[{"author_position":"first","author":{"id":"https://openalex.org/A5114234526","display_name":"Jagoda Rogowska","orcid":"https://orcid.org/0009-0004-5995-5802","observed_orcids":["https://orcid.org/0009-0004-5995-5802"]},"institutions":[{"id":"https://openalex.org/I4210122071","display_name":"Medical University of Lodz","ror":"https://ror.org/02t4ekc95","country_code":"PL","type":"education","lineage":["https://openalex.org/I4210122071"]}],"countries":["PL"],"is_corresponding":true,"raw_author_name":"Jagoda Rogowska","raw_affiliation_strings":["Department of Rheumatology, Medical University of Lodz, Lodz, Poland. jagoda.rogowska@umed.lodz.pl","International Doctoral School Medical University of Lodz, Lodz, Poland. jagoda.rogowska@umed.lodz.pl"],"raw_orcid":"https://orcid.org/0009-0004-5995-5802","affiliations":[{"raw_affiliation_string":"Department of Rheumatology, Medical University of Lodz, Lodz, Poland. jagoda.rogowska@umed.lodz.pl","institution_ids":["https://openalex.org/I4210122071"]},{"raw_affiliation_string":"International Doctoral School Medical University of Lodz, Lodz, Poland. jagoda.rogowska@umed.lodz.pl","institution_ids":["https://openalex.org/I4210122071"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5044956066","display_name":"Olga El\u017cbieta Brzezi\u0144ska","orcid":"https://orcid.org/0000-0002-3885-8497","observed_orcids":["https://orcid.org/0000-0002-3885-8497"]},"institutions":[{"id":"https://openalex.org/I4210122071","display_name":"Medical University of Lodz","ror":"https://ror.org/02t4ekc95","country_code":"PL","type":"education","lineage":["https://openalex.org/I4210122071"]}],"countries":["PL"],"is_corresponding":false,"raw_author_name":"Olga Brzezi\u0144ska","raw_affiliation_strings":["Department of Rheumatology, Medical University of Lodz, Lodz, Poland"],"raw_orcid":"https://orcid.org/0000-0002-3885-8497","affiliations":[{"raw_affiliation_string":"Department of Rheumatology, Medical University of Lodz, Lodz, Poland","institution_ids":["https://openalex.org/I4210122071"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5059575961","display_name":"Joanna Samanta Makowska","orcid":"https://orcid.org/0000-0003-2036-375X","observed_orcids":["https://orcid.org/0000-0003-2036-375X"]},"institutions":[{"id":"https://openalex.org/I4210122071","display_name":"Medical University of Lodz","ror":"https://ror.org/02t4ekc95","country_code":"PL","type":"education","lineage":["https://openalex.org/I4210122071"]}],"countries":["PL"],"is_corresponding":false,"raw_author_name":"Joanna Makowska","raw_affiliation_strings":["Department of Rheumatology, Medical University of Lodz, Lodz, Poland"],"raw_orcid":"https://orcid.org/0000-0003-2036-375X","affiliations":[{"raw_affiliation_string":"Department of Rheumatology, Medical University of Lodz, Lodz, Poland","institution_ids":["https://openalex.org/I4210122071"]}]},{"author_position":"last","author":{"id":"https://openalex.org/A5085265927","display_name":"Anna Lewandowska\u2010Polak","orcid":"https://orcid.org/0000-0002-8389-7399","observed_orcids":["https://orcid.org/0000-0002-8389-7399"]},"institutions":[{"id":"https://openalex.org/I4210122071","display_name":"Medical University of Lodz","ror":"https://ror.org/02t4ekc95","country_code":"PL","type":"education","lineage":["https://openalex.org/I4210122071"]}],"countries":["PL"],"is_corresponding":false,"raw_author_name":"Anna Lewandowska-Polak","raw_affiliation_strings":["Department of Rheumatology, Medical University of Lodz, Lodz, Poland"],"raw_orcid":"https://orcid.org/0000-0002-8389-7399","affiliations":[{"raw_affiliation_string":"Department of Rheumatology, Medical University of Lodz, Lodz, Poland","institution_ids":["https://openalex.org/I4210122071"]}]}],"institutions":[],"countries_distinct_count":1,"institutions_distinct_count":1,"corresponding_author_ids":["https://openalex.org/A5114234526"],"corresponding_institution_ids":["https://openalex.org/I4210122071"],"apc_list":{"value":4990,"currency":"USD","value_usd":4990},"apc_paid":{"value":4990,"currency":"USD","value_usd":4990},"fwci":0.0,"has_fulltext":true,"cited_by_count":0,"citation_normalized_percentile":{"value":0.0,"is_in_top_1_percent":false,"is_in_top_10_percent":false},"cited_by_percentile_year":null,"biblio":{"volume":"46","issue":"11","first_page":null,"last_page":null},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T11725","display_name":"Immunodeficiency and Autoimmune Disorders","score":1.0,"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"}},"topics":[{"id":"https://openalex.org/T11725","display_name":"Immunodeficiency and Autoimmune Disorders","score":1.0,"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/T10031","display_name":"T-cell and B-cell Immunology","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/T11616","display_name":"Autoimmune and Inflammatory Disorders Research","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/common-variable-immunodeficiency","display_name":"common variable immunodeficiency","score":0.9760000109672546},{"id":"https://openalex.org/keywords/rapamycin","display_name":"rapamycin","score":0.9829999804496765},{"id":"https://openalex.org/keywords/mtor-inhibition","display_name":"mTOR inhibition","score":0.7900000214576721},{"id":"https://openalex.org/keywords/immune-dysregulation","display_name":"immune dysregulation","score":0.9350000023841858},{"id":"https://openalex.org/keywords/hepatosplenomegaly","display_name":"hepatosplenomegaly","score":0.984000027179718},{"id":"https://openalex.org/keywords/lymphoproliferation","display_name":"lymphoproliferation","score":0.8799999952316284},{"id":"https://openalex.org/keywords/steroid-sparing-immunosuppression","display_name":"steroid-sparing immunosuppression","score":0.7419999837875366},{"id":"https://openalex.org/keywords/cytopenia","display_name":"cytopenia","score":0.7300000190734863},{"id":"https://openalex.org/keywords/platelet-responsiveness","display_name":"platelet response","score":0.75},{"id":"https://openalex.org/keywords/primary-immunodeficiency","display_name":"primary immunodeficiency","score":0.6549999713897705}],"concepts":[{"id":"https://openalex.org/C2779440204","wikidata":"https://www.wikidata.org/wiki/Q1260186","display_name":"Hepatosplenomegaly","level":3,"score":0.9571999907493591},{"id":"https://openalex.org/C2776433325","wikidata":"https://www.wikidata.org/wiki/Q1472818","display_name":"Common variable immunodeficiency","level":3,"score":0.907800018787384},{"id":"https://openalex.org/C71924100","wikidata":"https://www.wikidata.org/wiki/Q11190","display_name":"Medicine","level":0,"score":0.8827999830245972},{"id":"https://openalex.org/C2780194698","wikidata":"https://www.wikidata.org/wiki/Q6005429","display_name":"Immune dysregulation","level":3,"score":0.6717000007629395},{"id":"https://openalex.org/C203014093","wikidata":"https://www.wikidata.org/wiki/Q101929","display_name":"Immunology","level":1,"score":0.5931000113487244},{"id":"https://openalex.org/C126322002","wikidata":"https://www.wikidata.org/wiki/Q11180","display_name":"Internal medicine","level":1,"score":0.5450000166893005},{"id":"https://openalex.org/C2777921159","wikidata":"https://www.wikidata.org/wiki/Q32089","display_name":"Sirolimus","level":2,"score":0.388700008392334},{"id":"https://openalex.org/C8891405","wikidata":"https://www.wikidata.org/wiki/Q1059","display_name":"Immune system","level":2,"score":0.36239999532699585},{"id":"https://openalex.org/C90924648","wikidata":"https://www.wikidata.org/wiki/Q120569","display_name":"Gastroenterology","level":1,"score":0.34860000014305115},{"id":"https://openalex.org/C159654299","wikidata":"https://www.wikidata.org/wiki/Q79460","display_name":"Antibody","level":2,"score":0.33880001306533813},{"id":"https://openalex.org/C2779019163","wikidata":"https://www.wikidata.org/wiki/Q3043160","display_name":"Primary immunodeficiency","level":3,"score":0.31700000166893005},{"id":"https://openalex.org/C2777607303","wikidata":"https://www.wikidata.org/wiki/Q641307","display_name":"Immunodeficiency","level":3,"score":0.3073999881744385},{"id":"https://openalex.org/C2779102806","wikidata":"https://www.wikidata.org/wiki/Q79453","display_name":"Immunoglobulin A","level":4,"score":0.2985999882221222},{"id":"https://openalex.org/C2776553844","wikidata":"https://www.wikidata.org/wiki/Q79451","display_name":"Immunoglobulin M","level":4,"score":0.296999990940094},{"id":"https://openalex.org/C2780931953","wikidata":"https://www.wikidata.org/wiki/Q9371","display_name":"Spleen","level":2,"score":0.28279998898506165},{"id":"https://openalex.org/C2780898057","wikidata":"https://www.wikidata.org/wiki/Q79448","display_name":"Immunoglobulin G","level":3,"score":0.2809999883174896},{"id":"https://openalex.org/C2780653079","wikidata":"https://www.wikidata.org/wiki/Q412323","display_name":"Rituximab","level":3,"score":0.27810001373291016},{"id":"https://openalex.org/C2779969927","wikidata":"https://www.wikidata.org/wiki/Q916766","display_name":"Enzyme replacement therapy","level":3,"score":0.27079999446868896},{"id":"https://openalex.org/C198451711","wikidata":"https://www.wikidata.org/wiki/Q327657","display_name":"Rheumatology","level":2,"score":0.2623000144958496},{"id":"https://openalex.org/C2780252810","wikidata":"https://www.wikidata.org/wiki/Q1455316","display_name":"Immunosuppression","level":2,"score":0.2590000033378601}],"mesh":[{"descriptor_ui":"D000095542","descriptor_name":"Cytopenia","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":"D006529","descriptor_name":"Hepatomegaly","qualifier_ui":"Q000188","qualifier_name":"drug therapy","is_major_topic":true},{"descriptor_ui":"D006529","descriptor_name":"Hepatomegaly","qualifier_ui":"Q000209","qualifier_name":"etiology","is_major_topic":true},{"descriptor_ui":"D006529","descriptor_name":"Hepatomegaly","qualifier_ui":"Q000276","qualifier_name":"immunology","is_major_topic":true},{"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":true},{"descriptor_ui":"D008875","descriptor_name":"Middle Aged","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D013163","descriptor_name":"Splenomegaly","qualifier_ui":"Q000000981","qualifier_name":"diagnostic imaging","is_major_topic":true},{"descriptor_ui":"D013163","descriptor_name":"Splenomegaly","qualifier_ui":"Q000188","qualifier_name":"drug therapy","is_major_topic":true},{"descriptor_ui":"D013163","descriptor_name":"Splenomegaly","qualifier_ui":"Q000209","qualifier_name":"etiology","is_major_topic":true},{"descriptor_ui":"D013163","descriptor_name":"Splenomegaly","qualifier_ui":"Q000276","qualifier_name":"immunology","is_major_topic":true},{"descriptor_ui":"D016896","descriptor_name":"Treatment Outcome","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D017074","descriptor_name":"Common Variable Immunodeficiency","qualifier_ui":"Q000150","qualifier_name":"complications","is_major_topic":true},{"descriptor_ui":"D017074","descriptor_name":"Common Variable Immunodeficiency","qualifier_ui":"Q000188","qualifier_name":"drug therapy","is_major_topic":true},{"descriptor_ui":"D017074","descriptor_name":"Common Variable Immunodeficiency","qualifier_ui":"Q000276","qualifier_name":"immunology","is_major_topic":true},{"descriptor_ui":"D020123","descriptor_name":"Sirolimus","qualifier_ui":"Q000627","qualifier_name":"therapeutic use","is_major_topic":true}],"locations_count":3,"locations":[{"id":"doi:10.1007/s00296-026-06329-4","doi":"https://doi.org/10.1007/s00296-026-06329-4","is_oa":true,"landing_page_url":"https://doi.org/10.1007/s00296-026-06329-4","pdf_url":"https://link.springer.com/content/pdf/10.1007/s00296-026-06329-4.pdf","source":{"id":"https://openalex.org/S50823678","display_name":"Rheumatology International","issn_l":"0172-8172","issn":["0172-8172","1437-160X"],"is_oa":false,"is_in_doaj":false,"is_core":true,"listed_in":["anvur-scientific","cwts-core","dongbi-d","doyens","jufo-1","ki-jl-1","medline","norway-1","poland-70","russia-white-list-2","vabb-shw"],"host_organization":"https://openalex.org/P4310319900","host_organization_name":"Springer Science+Business Media","host_organization_lineage":["https://openalex.org/P4310319900","https://openalex.org/P4310319965"],"host_organization_lineage_names":["Springer Science+Business Media","Springer Nature"],"type":"journal"},"license":"cc-by-nc-nd","license_id":"https://openalex.org/licenses/cc-by-nc-nd","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Rheumatology International","raw_type":"journal-article"},{"id":"pmh:oai:pubmedcentral.nih.gov:13740097","doi":null,"is_oa":true,"landing_page_url":"https://pmc.ncbi.nlm.nih.gov/articles/PMC13740097/","pdf_url":"https://pmc.ncbi.nlm.nih.gov/articles/PMC13740097/pdf/296_2026_Article_6329.pdf","source":{"id":"https://openalex.org/S2764455111","display_name":"PubMed Central","issn_l":null,"issn":null,"is_oa":true,"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":"cc-by-nc-nd","license_id":"https://openalex.org/licenses/cc-by-nc-nd","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Rheumatol Int","raw_type":"Text"},{"id":"pmid:42986447","doi":null,"is_oa":false,"landing_page_url":"https://pubmed.ncbi.nlm.nih.gov/42986447","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":"Rheumatology international","raw_type":"Journal Article"}],"best_oa_location":{"id":"doi:10.1007/s00296-026-06329-4","doi":"https://doi.org/10.1007/s00296-026-06329-4","is_oa":true,"landing_page_url":"https://doi.org/10.1007/s00296-026-06329-4","pdf_url":"https://link.springer.com/content/pdf/10.1007/s00296-026-06329-4.pdf","source":{"id":"https://openalex.org/S50823678","display_name":"Rheumatology International","issn_l":"0172-8172","issn":["0172-8172","1437-160X"],"is_oa":false,"is_in_doaj":false,"is_core":true,"listed_in":["anvur-scientific","cwts-core","dongbi-d","doyens","jufo-1","ki-jl-1","medline","norway-1","poland-70","russia-white-list-2","vabb-shw"],"host_organization":"https://openalex.org/P4310319900","host_organization_name":"Springer Science+Business Media","host_organization_lineage":["https://openalex.org/P4310319900","https://openalex.org/P4310319965"],"host_organization_lineage_names":["Springer Science+Business Media","Springer Nature"],"type":"journal"},"license":"cc-by-nc-nd","license_id":"https://openalex.org/licenses/cc-by-nc-nd","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Rheumatology International","raw_type":"journal-article"},"sustainable_development_goals":[{"id":"https://metadata.un.org/sdg/3","score":1.0,"display_name":"Good health and well-being"}],"study_designs":[{"id":"https://openalex.org/study-designs/case-report","display_name":"Case Report"}],"awards":[],"funders":[{"id":"https://openalex.org/F4320323344","display_name":"Uniwersytet \u0141\u00f3dzki","ror":"https://ror.org/05cq64r17"}],"has_content":{"grobid_xml":true,"pdf":true},"content_urls":{"pdf":"https://content.openalex.org/works/W7225340303.pdf","grobid_xml":"https://content.openalex.org/works/W7225340303.grobid-xml"},"referenced_works_count":21,"referenced_works":["https://openalex.org/W1971833928","https://openalex.org/W2050278963","https://openalex.org/W2057916571","https://openalex.org/W2120048822","https://openalex.org/W2590609776","https://openalex.org/W2788578651","https://openalex.org/W2890806955","https://openalex.org/W3149316490","https://openalex.org/W4306878786","https://openalex.org/W4309162882","https://openalex.org/W4367050849","https://openalex.org/W4385655375","https://openalex.org/W4385786424","https://openalex.org/W4386895591","https://openalex.org/W4386985621","https://openalex.org/W4389781886","https://openalex.org/W4390012990","https://openalex.org/W4400598644","https://openalex.org/W4411827678","https://openalex.org/W7127432833","https://openalex.org/W7165761589"],"related_works":["https://openalex.org/W2171290245","https://openalex.org/W2468819310","https://openalex.org/W7162605233","https://openalex.org/W2742685198","https://openalex.org/W3208739699","https://openalex.org/W2255251553","https://openalex.org/W3007427944","https://openalex.org/W4417201999","https://openalex.org/W4390949820","https://openalex.org/W2093766132"],"abstract_inverted_index":{"Common":[0],"variable":[1],"immunodeficiency":[2],"(CVID)":[3],"is":[4,35,51],"the":[5,79],"most":[6],"common":[7],"primary":[8],"immune":[9,46,169,287],"defficiency":[10],"in":[11,58,245,271,281,300],"adults.":[12],"Patients":[13],"with":[14,17,66,86,223,285],"CVID":[15,71,87],"present":[16],"predominant":[18],"antibody":[19],"deficiency":[20],"and":[21,48,68,72,101,115,123,142,188,213,234,247,264,289,296],"reduced":[22],"serum":[23],"levels":[24],"of":[25,64,81,154],"immunoglobulin":[26],"class":[27],"G,":[28],"A":[29],"and/":[30],"or":[31],"M.":[32],"The":[33,108,241],"disease":[34],"frequently":[36],"complicated":[37,272],"by":[38],"non-infectious":[39],"organ":[40,262],"involvement,":[41,292],"including":[42,70],"hepatosplenomegaly":[43,100,148],"secondary":[44],"to":[45,129,158,182,201,209,220,229,269],"dysregulation":[47,288],"lymphoproliferation.":[49],"Sirolimus":[50,160],"a":[52,82,164,260,265],"mTORC1":[53],"inhibitor,":[54],"used":[55],"as":[56,163],"immunosuppressant":[57],"transplant":[59],"medicine,":[60],"increasingly":[61],"inborn":[62],"errors":[63],"immunity":[65],"lymphoproliferation":[67,156],"autoimmunity,":[69],"monogenic":[73],"CVID-like":[74],"disorders.":[75],"Therefore,":[76],"we":[77],"describe":[78],"case":[80,258],"50-year-old":[83],"patient":[84,109,242],"diagnosed":[85],"for":[88,167],"5":[89,186],"years,":[90],"on":[91],"regular":[92],"IVIG":[93],"(intravenous":[94],"immunoglobulins)":[95],"replacement":[96],"who":[97],"developed":[98],"progressive":[99],"cytopenias":[102],"despite":[103],"adequate":[104],"infection":[105,141],"preventive":[106],"healthcare.":[107],"reported":[110,243],"increasing":[111],"abdominal":[112,248],"enlargement,":[113],"dyspnea,":[114],"fatigue.":[116],"She":[117],"had":[118],"also":[119],"noted":[120],"excessive":[121],"daytime":[122],"nighttime":[124],"sweating,":[125],"initially":[126],"partly":[127],"attributed":[128],"menopausal":[130],"status.":[131],"Physical":[132],"examination":[133],"confirmed":[134],"marked":[135,261],"hepatosplenomegaly.":[136],"Initial":[137],"evaluation":[138],"excluded":[139],"acute":[140],"overt":[143],"lymphoma.":[144],"Abdominal":[145],"imaging":[146],"revealed":[147],"without":[149],"focal":[150],"lesions,":[151],"raising":[152],"suspicion":[153],"immune-mediated":[155],"related":[157],"CVID.":[159],"was":[161],"introduced":[162],"steroid-sparing":[165],"agent":[166],"presumed":[168],"dysregulation.":[170],"During":[171],"sirolimus":[172,270],"treatment,":[173],"spleen":[174],"length":[175],"decreased":[176],"from":[177,198,207,218,227],"23":[178],"cm":[179,184,190,200],"before":[180],"initiation":[181],"13":[183],"after":[185,191],"months":[187],"12":[189],"7":[192],"months,":[193],"while":[194],"liver":[195],"size":[196],"fell":[197],"18":[199],"15":[202],"cm.":[203],"Platelet":[204],"count":[205],"rose":[206],"37":[208],"75":[210],"\u00d7":[211],"10\u00b3/\u00b5L,":[212],"aspartate":[214],"aminotransferase":[215,225],"activity":[216],"declined":[217],"77":[219],"58":[221],"U/L,":[222],"alanine":[224],"dropping":[226],"93":[228],"47":[230],"U/L.":[231],"Leukopenia,":[232],"neutropenia,":[233],"elevated":[235],"alkaline":[236],"phosphatase":[237],"activity,":[238],"however,":[239],"persisted.":[240],"improvement":[244],"constitutional":[246],"symptoms,and":[249],"no":[250],"serious":[251],"infectious":[252],"complications":[253],"occurred":[254],"during":[255],"treatment.":[256],"This":[257],"demonstrates":[259],"response":[263,268],"partial":[266],"platelet":[267],"CVID,":[273],"suggesting":[274],"that":[275],"mTOR":[276],"inhibition":[277],"may":[278],"be":[279],"considered":[280],"carefully":[282],"selected":[283],"patients":[284],"CVID-associated":[286],"lymphoproliferative":[290],"hepatosplenic":[291],"although":[293],"its":[294],"efficacy":[295],"safety":[297],"require":[298],"confirmation":[299],"larger":[301],"studies.":[302]},"counts_by_year":[],"updated_date":"2026-11-10T11:39:43.021295","created_date":"2026-11-06T00:00:00"}
