Table 1.
Characteristics and outcome of treatment with LowR-BD in patients with wAIHA.
Patient | Sex | Age (years) | Diagnosis | Disease duration | Previous treatment | HGB (g/L) before treatment | RBC (∗1012/L) before treatment | Ret (%) before treatment | Transfusion during previous treatment (U) | Days to elevation of HGB > 20 g/L | LowR-BD treatment cycle | Transfusion during LowR-BD treatment (U) | Response after LowR-BD | Time to PR (days) | Time to CR (days) | Follow-up |
1 | F | 65 | Evans syndrome, SLE, wAIHA | 18 mo | GCs | 64 | 2.23 | 5.03 | 0 | 10 | 4 | 2 | PR | 10 | 24 | CCR |
2 | F | 61 | wAIHA | 2 mo | FD, PE | 63 | 1.76 | 10.83 | 0 | 9 | 4 | 4 | CR | 9 | 11 | CCR |
3 | F | 48 | RA, MBL, wAIHA | 1 mo | GCs | 54 | 1.86 | 8.61 | 0 | 8 | 4 | 4 | CR | 8 | 28 | CCR |
4 | M | 39 | wAIHA | 2 d | FD, PE | 58 | 1.82 | 13.32 | 0 | 7 | 4 | 4 | CR | 7 | 15 | CCR |
5 | M | 74 | wAIHA | 15 d | GCs | 50 | 1.15 | 23.22 | 4 | 23 | 4 | 0 | NR | 95 | 233 | Reached CR after using CYC for 6. 5 mo |
6 | F | 48 | wAIHA, WM | 6 yr | COP, Spl, GCs, Rtx, PE, CYC | 47 | 1.08 | 14.69 | 18 | 14 | 4 | 4 | CR | 14 | 21 | Relapse 10 mo later, PR was achieved after treatment with 2 cycles of LowR-BD. |
7 | M | 70 | wAIHA, SS | 9 mo | GCs, HCQ, TGs, PE, CsA, Rtx, MMF | 47 | 1.05 | 7.61 | 12 | 18 | 3 | 12 + 4 | PR | 26 | – | PR |
CCR = continuous complete response, COP = cyclophosphamide + vincristine + prednisone, CR = complete response, CsA = ciclosporin, CYC = cyclophosphamide, F = female, FD = first diagnosis, GCs = glucocorticoids, HCQ = hydroxychloroquine, HGB = haemoglobin, LowR-BD = rituximab + bortezomib + dexamethasone, M = male, MBL = Monoclonal B-cell lymphocytosis, MMF = mycophenolate mofetil, PE = plasma exchange, PR = partial response, RA = rheumatoid arthritis, RBC = red blood cell, Ret = reticulocyte, Rtx = rituximab, SLE = systemic lupus erythematous, Spl = splenectomy, SS = Sjogrensyndrome, TGs = Tripterygium glycosides, wAIHA = warm autoimmune hemolytic anemia, WM = waldenstrom macroglobulinaemia.