From: Active IgG4-related disease with bone marrow involvement: a report of 2 cases and case-based review
No. | Author | Age range (years) | Race, gender | Extra-hematologic organs involved in IgG4-RD | Presentation of hematologic involvement | Detection of bone marrow involvement | Treatment | Prognosis |
---|---|---|---|---|---|---|---|---|
1 | Kim [12] | 60–65 | Korean, M | Kidney: IgG4-related tubulointerstitial nephritis Skin: rash (possible associated with eosinophilia) | Multiple lymphadenopathies; leukocytosis, thrombocytopenia, marked eosinophilia (51%), and rare nucleated red blood cells (1/100 WBCs) | FDG-PET/CT: hyperactive BM; BM biopsy: 20.4% eosinophils; 16.8% immature plasma cells; increased numbers of CD138-positive plasma cells; IgG4/IgG-positive plasma cell ratio = 0.88 PB: 58.3% of plasma cells were atypical (low CD19 expression with CD38 + /CD138dim/CD56 − /CD45 +  | High-dose steroids | Symptoms improved |
2 | Ichiki [7] | 60–65 | Japanese, F | Submandibular gland swelling | Anemia, IgG4-related lymphadenopathies by lymph node biopsy | FDG-PET/CT: uptakes in multiple bones and immunohistochemically proven by BM biopsy BM biopsy: > 50% of CD138-positive plasma cells were IgG4 positive | Steroids 0.5 mg/kg | Anemia and submandibular gland swelling improved |
3 | van den Elshout-den Uyl [10] | 75–80 | Netherland, M | Fatigue, increased exhaustion after physical exercise, weight loss, and night sweats | Anemia and leucocytosis | MRI: showed multiple hypodense bone lesions FDG-PET/CT: FDG-avid bone lesions along the spinal cord BM biopsy: < 10% plasma cells in pre-existing bone marrow, but around 25% plasma cells were present in the fibrotic lesion | Prednisolone starting dose 40 mg/day | Symptoms improved, as well as serum hemoglobin and ESR |
4 | Tarte [33] | 40–45 | African-American, M | Kidneys (TIN, imaging abnormalities), lungs, liver, and small intestine (imaging abnormalities) | Lymphadenopathies (biopsy-proven), anemia and thrombocytopenia | BM biopsy: mild plasmacytosis (10–15% of total cellularity). a subset of plasma cells exhibited specificity for IgG4 + stain (< 20% of total IgG + plasma cells) | Prednisone 60 mg/day, Rituximab | Symptoms improved |
5 |  | 65–70 | Chinese, M | Kidneys (IgG4-TIN, imaging abnormalities), submaxillary glands, arteritis | Leukopenia, anemia and thrombocytopenia, lymphadenopathies | BM biopsy: 50% of CD138-positive plasma cells were IgG4-positive, scattered eosinophils | Prednisone 40 mg/day | Symptoms improved |
6 |  | 35–40 | Chinese, M | Kidneys (IgG4-TIN), submandibular gland | Marked eosinophilia (15%), lymphadenopathies | FDG-PET/CT: hyperactive BM | Prednisone 50 mg/day, Rituximab | Symptoms improved |