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Table 2 Literature review of bone marrow involvement in IgG4-RD

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

  1. 5 and 6 are our presented cases
  2. PB peripheral blood, BM bone marrow, MRI magnetic resonance imaging, TIN tubulointerstitial nephritis