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Classic hematologic presentations including lymphadenopathy, eosinophilia and polyclonal hypergammaglobulinemia are common, whereas bone marrow involvement of IgG4-RD is rarely reported. Here we present two patients of multi-organ IgG4-RD with bone marrow involvement, one on bone marrow biopsy, and the other on PET\/CT. Presentations of other organ involvement included biopsy-proven IgG4-related tubulointerstitial nephritis, lymphadenopathies, submaxillary glands, arteritis, asthma, dysosmia, and constitutional symptoms. Bone marrow involvement was initially suspected due to leukopenia, anemia and thrombocytopenia in case#1, and was finally confirmed by histological evidence of significant IgG4-positive plasma cells infiltration in bone marrow. In case#2, we incidentally observed high uptakes of multi-bone marrow on <jats:sup>18<\/jats:sup>F-FDG-PET\/CT, with the maximum SUV value similar to that of the kidneys, submaxillary glands and hilar, mediastinal lymph nodes by <jats:sup>18<\/jats:sup>F-FDG-PET\/CT. Symptoms and all the hematologic presentation improved rapidly in both patients after steroids initiation. These two cases illustrate the rare bone marrow involvement in active IgG4-RD accompanied by other hematologic syndromes. The significance of disease pathogenesis is worthy of further study.<\/jats:p>","DOI":"10.1186\/s40001-022-00643-w","type":"journal-article","created":{"date-parts":[[2022,2,2]],"date-time":"2022-02-02T10:02:59Z","timestamp":1643796179000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Active IgG4-related disease with bone marrow involvement: a report of 2 cases and case-based review"],"prefix":"10.1186","volume":"27","author":[{"given":"Shiyi","family":"Liu","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hui","family":"Wang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6857-8146","authenticated-orcid":false,"given":"Tao","family":"Su","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2022,2,2]]},"reference":[{"issue":"9976","key":"643_CR1","doi-asserted-by":"publisher","first-page":"1460","DOI":"10.1016\/S0140-6736(14)60720-0","volume":"385","author":"T Kamisawa","year":"2015","unstructured":"Kamisawa T, Zen Y, Pillai S, Stone HJ. 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