Table 2 Clinical features of TAFRO syndrome in eight previously reported cases and in our case
Case no. | Age/sex | Thrombocytopenia (PLT ×104/μl) | Anasarca | Reticulin fibrosis of the bone marrow | Renal dysfunction | Organomegaly | Hb (g/dl) | IgG (mg/dl) | Serum IL-6 (pg/ml) (normal range) | Plasma VEGF (pg/ml) (normal range) | Lymph node biopsy | Treatment | Outcome | Ref |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
1 | 47/F | 1.5 | + | + | No data | + | 10.9 | 1046 | No data | 285 (<115) | No data | CHOP, PSL | Survival | [6] |
2 | 56/M | 1.9 | + | + | + | + | 10.7 | 863 | 7.2 (<4) | 31 (<115) | No data | PSL, IVIG, CyA | Survival | |
3 | 49/M | 1.0 | + | + | No data | + | 11.7 | 1057 | 64.9 (<4) | 104 (<115) | HV-type of CD | PSL, IVIG | Death (MOF) | |
4 | 49/F | 1.7 | + | + | + | + | 6.2 | 2611 | 11 (No data) | 330 (No data) | unclear | DEX, PSL, CyA | Survival | [2] |
5 | 43/F | <3.0 | + | No data | + | + | 12.6 | 965 | 45.6 (<4) | 665 (<115) | HV-type of CD | mPSL, PSL, RTX, TCZ | Survival | [3] |
6 | 47/F | 3.9 | + | + | + | + | 9.1 | 1426 | 21.9 (No data) | No data | PC-type of CD | PSL, mPSL, TCZ | Survival | [4] |
7 | 57/F | 1.3 | + | - | + | + | 7.1 | 1860 | 65.5 (No data) | 91 (No data) | unclear | mPSL, PSL, CHOEP | Death (Sepsis) | [5] |
8 | 73/M | 2.4 | + | + | + | + | 7.6 | 7080 | 49 (No data) | 25 (No data) | Mixed type of CD | mPSL, PSL | Death (MOF) | |
Current case | 15/M | 3.0 | + | + | + | + | 7.2 | 729 | 29 (<3) | 178 (<38.3) | Mixed type of CD | mPSL, PSL, TCZ, IVIG | Survival |