{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,23]],"date-time":"2026-09-23T18:13:20Z","timestamp":1790187200878,"version":"4.1.0"},"reference-count":19,"publisher":"Oxford University Press (OUP)","issue":"1","license":[{"start":{"date-parts":[[2026,1,30]],"date-time":"2026-01-30T00:00:00Z","timestamp":1769731200000},"content-version":"vor","delay-in-days":29,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2026,1,6]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Haematological manifestations of immunoglobulin (Ig)G4-related disease (IgG4-RD) are atypical, and few studies have addressed cytopenia in this condition. We present here a case of IgG4-RD with marked pancytopenia and splenomegaly in the absence of bone marrow abnormalities. A 67-year-old woman developed diplopia, dyspnoea, pancytopenia, and hypocomplementemia. Contrast-enhanced computed tomography showed bilateral lacrimal and submandibular gland swelling, enlargement of the cervical, mediastinal, inguinal, and para-aortic lymph nodes, splenomegaly, and granular shadows in both lungs. She had been referred to our hospital on suspicion of malignant lymphoma, but a lymph node biopsy indicated the possibility of IgG4-RD. A lacrimal gland biopsy showed infiltration of numerous lymphocytes and IgG4-positive plasma cells along with mild fibrosis. A bone marrow biopsy showed normocellular marrow with no increase in plasma cells. The IgG4-positive plasma cell count was 4 per high-power field, with an IgG4\/IgG ratio of 10%. After excluding potential mimics, the diagnosis was IgG4-RD presenting with pancytopenia and splenomegaly. Prednisolone was initiated at 40\u00a0mg\/day, leading to the rapid normalisation of pancytopenia, recovery of complement levels, and resolution of other organ involvement such as splenomegaly. Physicians should keep in mind that IgG4-RD can present with pancytopenia and splenomegaly.<\/jats:p>","DOI":"10.1093\/mrcr\/rxag008","type":"journal-article","created":{"date-parts":[[2026,1,28]],"date-time":"2026-01-28T12:38:20Z","timestamp":1769603900000},"source":"Crossref","is-referenced-by-count":1,"title":["Pancytopenia and splenomegaly as uncommon manifestations in a patient with immunoglobulin G4-related disease"],"prefix":"10.1093","volume":"10","author":[{"ORCID":"https:\/\/orcid.org\/0009-0001-2356-7101","authenticated-orcid":false,"given":"Kojiro","family":"Ikeda","sequence":"first","affiliation":[{"name":"Department of Rheumatology, University of Yamanashi Hospital , Yamanashi ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8964-8513","authenticated-orcid":false,"given":"Shunichiro","family":"Hanai","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, University of Yamanashi Hospital , Yamanashi ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0004-8746-3901","authenticated-orcid":false,"given":"Soichiro","family":"Kubota","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, University of Yamanashi Hospital , Yamanashi ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0005-3929-4215","authenticated-orcid":false,"given":"Nakako","family":"Tanaka-Mabuchi","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, University of Yamanashi Hospital , Yamanashi ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0002-0780-9868","authenticated-orcid":false,"given":"Mayu","family":"Yazaki","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, University of Yamanashi Hospital , Yamanashi ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1889-2855","authenticated-orcid":false,"given":"Yoshiaki","family":"Kobayashi","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, University of Yamanashi Hospital , Yamanashi ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1682-9799","authenticated-orcid":false,"given":"Daiki","family":"Nakagomi","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, University of Yamanashi Hospital , Yamanashi ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2026,1,30]]},"reference":[{"key":"2026081301164261600_ref1","doi-asserted-by":"crossref","first-page":"1","DOI":"10.3109\/s10165-011-0508-6","article-title":"A novel clinical entity, IgG4-related disease (IgG4RD): general concept and details","volume":"22","author":"Umehara","year":"2012","journal-title":"Mod Rheumatol"},{"key":"2026081301164261600_ref2","doi-asserted-by":"crossref","first-page":"1911","DOI":"10.2169\/internalmedicine.0190-17","article-title":"Concurrent autoimmune neutropenia and idiopathic thrombocytopenic purpura associated with IgG4-related disease","volume":"57","author":"Shimazu","year":"2018","journal-title":"Intern Med"},{"key":"2026081301164261600_ref3","doi-asserted-by":"crossref","DOI":"10.1136\/bcr-2018-224814","article-title":"IgG4-related disease coexisting with autoimmune haemolytic anaemia","volume":"2018","author":"Wang","year":"2018","journal-title":"BMJ Case Rep"},{"key":"2026081301164261600_ref4","doi-asserted-by":"crossref","first-page":"77","DOI":"10.1136\/annrheumdis-2019-216561","article-title":"The 2019 American College of Rheumatology\/European league against rheumatism classification criteria for IgG4-related disease","volume":"79","author":"Wallace","year":"2020","journal-title":"Ann Rheum Dis"},{"key":"2026081301164261600_ref5","doi-asserted-by":"crossref","first-page":"444","DOI":"10.3324\/haematol.2018.205526","article-title":"IgG4-related disease: what a hematologist needs to know","volume":"104","author":"Chen","year":"2019","journal-title":"Haematologica."},{"key":"2026081301164261600_ref6","doi-asserted-by":"crossref","first-page":"391","DOI":"10.1111\/pin.12932","article-title":"Clinicopathological differential diagnosis of IgG4-related disease: a historical overview and a proposal of the criteria for excluding mimickers of IgG4-related disease","volume":"70","author":"Satou","year":"2020","journal-title":"Pathol Int"},{"key":"2026081301164261600_ref7","first-page":"392893","article-title":"A case of immunoglobulin G4-related disease with extensive multiorgan involvements","volume":"2015","author":"Higashioka","year":"2015","journal-title":"Case Rep Rheumatol"},{"key":"2026081301164261600_ref8","doi-asserted-by":"crossref","first-page":"361","DOI":"10.1016\/j.blre.2018.03.001","article-title":"Approach to pancytopenia: diagnostic algorithm for clinical hematologists","volume":"32","author":"Gnanaraj","year":"2018","journal-title":"Blood Rev"},{"key":"2026081301164261600_ref9","doi-asserted-by":"crossref","first-page":"647","DOI":"10.1056\/NEJMcp1900554","article-title":"Warm autoimmune hemolytic anemia","volume":"381","author":"Brodsky","year":"2019","journal-title":"N Engl J Med"},{"key":"2026081301164261600_ref10","doi-asserted-by":"crossref","first-page":"520","DOI":"10.3389\/fimmu.2014.00520","article-title":"IgG subclasses and allotypes: from structure to effector functions","volume":"5","author":"Vidarsson","year":"2014","journal-title":"Front Immunol"},{"key":"2026081301164261600_ref11","doi-asserted-by":"crossref","first-page":"275","DOI":"10.1038\/s41584-025-01240-x","article-title":"IgG4-related disease and other fibro-inflammatory conditions","volume":"21","author":"Peyronel","year":"2025","journal-title":"Nat Rev Rheumatol"},{"key":"2026081301164261600_ref12","doi-asserted-by":"crossref","first-page":"1773","DOI":"10.1136\/ard-2024-225846","article-title":"Multiorgan involvement and circulating IgG1 predict hypocomplementaemia in IgG4-related disease","volume":"83","author":"Katz","year":"2024","journal-title":"Ann Rheum Dis"},{"key":"2026081301164261600_ref13","doi-asserted-by":"crossref","first-page":"2865","DOI":"10.3892\/etm.2016.3692","article-title":"Hypersplenism: history and current status","volume":"12","author":"Lv","year":"2016","journal-title":"Exp Ther Med"},{"key":"2026081301164261600_ref14","doi-asserted-by":"crossref","first-page":"643","DOI":"10.1016\/j.gtc.2018.04.009","article-title":"Splenomegaly a combined clinical and radiologic approach to the differential diagnosis","volume":"47","author":"Sjoberg","year":"2018","journal-title":"Gastroenterol Clin N Am"},{"key":"2026081301164261600_ref15","doi-asserted-by":"crossref","first-page":"2295","DOI":"10.2169\/internalmedicine.55.6749","article-title":"IgG4-related disease with bone marrow involvement","volume":"55","author":"Ichiki","year":"2016","journal-title":"Intern Med"},{"key":"2026081301164261600_ref16","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1186\/s40001-022-00643-w","article-title":"Active IgG4-related disease with bone marrow involvement: a report of 2 cases and case-based review","volume":"27","author":"Liu","year":"2022","journal-title":"Eur J Med Res"},{"key":"2026081301164261600_ref17","doi-asserted-by":"crossref","first-page":"549","DOI":"10.1007\/s00296-023-05510-3","article-title":"An overlapping case of IgG4-related disease and systemic lupus erythematosus treated with belimumab: a case-based review","volume":"44","author":"Iwamoto","year":"2024","journal-title":"Rheumatol Int"},{"key":"2026081301164261600_ref18","doi-asserted-by":"crossref","first-page":"e14429","DOI":"10.1111\/nep.14429","article-title":"A challenging case of IgG4-related kidney disease accompanied with positive serologic tests of lupus erythematosus","volume":"30","author":"Jeerangsapasuk","year":"2025","journal-title":"Nephrology."},{"key":"2026081301164261600_ref19","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.2025.287794","article-title":"Serial next-generation sequencing for detecting germline predisposition in acute myeloid leukemia","author":"Ahn","journal-title":"Haematologica."}],"container-title":["Modern Rheumatology Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/mrcr\/advance-article-pdf\/doi\/10.1093\/mrcr\/rxag008\/66640811\/rxag008.pdf","content-type":"application\/pdf","content-version":"am","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/mrcr\/article-pdf\/10\/1\/rxag008\/66640811\/rxag008.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/mrcr\/article-pdf\/10\/1\/rxag008\/66640811\/rxag008.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,8,13]],"date-time":"2026-08-13T05:16:49Z","timestamp":1786598209000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/mrcr\/article\/doi\/10.1093\/mrcr\/rxag008\/8445423"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026]]},"references-count":19,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2026,1,6]]}},"URL":"https:\/\/doi.org\/10.1093\/mrcr\/rxag008","relation":{},"ISSN":["2472-5625"],"issn-type":[{"value":"2472-5625","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2026]]},"published":{"date-parts":[[2026]]},"article-number":"rxag008"}}