Active IgG4-related disease with bone marrow involvement: a report of 2 cases and case-based review

Abstract

IgG4-related disease (IgG4-RD) is a systemic fibro-inflammatory disease, histopathologically characterized by dense lymphoplasmacytic infiltration rich in IgG4-positive plasma cells in affected organs. 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 18F-FDG-PET/CT, with the maximum SUV value similar to that of the kidneys, submaxillary glands and hilar, mediastinal lymph nodes by 18F-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.