Treatments received and efficacy and safety according to the cause of CryoVas, the therapeutic regimen used, and the line of treatment
| . | Causative factors . | Efficacy, n/N (%) . | Tolerance, n/N (%) . | |||||||
|---|---|---|---|---|---|---|---|---|---|---|
| All patients . | Essential MC . | Connective tissue . | Hematologic disease . | Complete clinical response* . | Renal response† . | Immunologic response‡ . | Refractory disease§ . | Severe infection . | Death . | |
| No. | 209 | 92 | 71 | 46 | ||||||
| CTC alone | ||||||||||
| First-line therapy, n | 103 | 48 | 47 | 8 | 45/103 (44) | 14/23 (61) | 25/76 (33) | 29/103 (29) | 11/103 (11) | 9/103 (9) |
| CTC, mg/d, median dosage (range) | 50 (10-180) | 55 (10-180) | 40 (15-80) | 55 (15-120) | ||||||
| ≥ Second-line therapy, n | 45 | 23 | 17 | 5 | 15/45 (33) | 10/17 (59) | 17/38 (45) | 12/45 (27) | 5/45 (11) | 6/45 (13) |
| CTC, mg/d, median dosage (range) | 40 (10-80) | 40 (10-80) | 40 (15-60) | 50 (20-80) | ||||||
| Alkylating agents plus CTC | ||||||||||
| First-line therapy, n‖ | 55 | 24 | 16 | 15 | 34/55 (62) | 25/29 (86) | 32/44 (73) | 6/55 (11) | 5/55 (9) | 7/55 (13) |
| CTC, mg/d, median dosage (range) | 60 (20-120) | 60 (40-100) | 50 (30-70) | 50 (20-120) | ||||||
| ≥ Second-line therapy, n¶ | 28 | 17 | 9 | 2 | 11/28 (39) | 8/12 (66) | 11/23 (48) | 6/28 (21) | 2/28 (7)) | 1/28 (4) |
| CTC, mg/d, median dosage (range) | 50 (7-70) | 50 (10-70) | 45 (7-60) | 20 (20-20) | ||||||
| Rituximab plus CTC | ||||||||||
| First-line therapy, n# | 28 | 14 | 4 | 10 | 18/28 (64) | 11/13 (85) | 17/22 (77) | 3/28 (11) | 8/28 (29) | 3/28 (11) |
| CTC, mg/d, median dosage (range) | 60 (20-80) | 50 (20-80) | 40 (20-60) | 60 (50-70) | ||||||
| ≥ Second-line therapy, n** | 58 | 23 | 29 | 6 | 38/58 (66) | 15/17 (88) | 41/49 (84) | 3/58 (5) | 11/58 (19) | 5/58 (9) |
| CTC, mg/d, median dosage (range) | 40 (5-80) | 50 (10-80) | 40 (5-80) | 22.5 (15-40) | ||||||
| Rituximab plus alkylating agents regimen | ||||||||||
| First-line therapy, n†† | 12 | 1 | 0 | 11 | 10/12 (83) | 7/7 (100) | 8/10 (80) | 0/12 (0) | 5/12 (42) | 3/12 (25) |
| ≥ Second-line therapy, n‡‡ | 8 | 1 | 1 | 6 | 3/8 (38) | 2/5 (40) | 2/7 (29) | 3/8 (38) | 1/8 (13) | 1/8 (13) |
| Alternative therapy | ||||||||||
| First-line therapy, n | 11 | 5 | 4 | 2 | ||||||
| ≥ Second-line therapy, n | 25 | 9 | 12 | 4 | ||||||
| . | Causative factors . | Efficacy, n/N (%) . | Tolerance, n/N (%) . | |||||||
|---|---|---|---|---|---|---|---|---|---|---|
| All patients . | Essential MC . | Connective tissue . | Hematologic disease . | Complete clinical response* . | Renal response† . | Immunologic response‡ . | Refractory disease§ . | Severe infection . | Death . | |
| No. | 209 | 92 | 71 | 46 | ||||||
| CTC alone | ||||||||||
| First-line therapy, n | 103 | 48 | 47 | 8 | 45/103 (44) | 14/23 (61) | 25/76 (33) | 29/103 (29) | 11/103 (11) | 9/103 (9) |
| CTC, mg/d, median dosage (range) | 50 (10-180) | 55 (10-180) | 40 (15-80) | 55 (15-120) | ||||||
| ≥ Second-line therapy, n | 45 | 23 | 17 | 5 | 15/45 (33) | 10/17 (59) | 17/38 (45) | 12/45 (27) | 5/45 (11) | 6/45 (13) |
| CTC, mg/d, median dosage (range) | 40 (10-80) | 40 (10-80) | 40 (15-60) | 50 (20-80) | ||||||
| Alkylating agents plus CTC | ||||||||||
| First-line therapy, n‖ | 55 | 24 | 16 | 15 | 34/55 (62) | 25/29 (86) | 32/44 (73) | 6/55 (11) | 5/55 (9) | 7/55 (13) |
| CTC, mg/d, median dosage (range) | 60 (20-120) | 60 (40-100) | 50 (30-70) | 50 (20-120) | ||||||
| ≥ Second-line therapy, n¶ | 28 | 17 | 9 | 2 | 11/28 (39) | 8/12 (66) | 11/23 (48) | 6/28 (21) | 2/28 (7)) | 1/28 (4) |
| CTC, mg/d, median dosage (range) | 50 (7-70) | 50 (10-70) | 45 (7-60) | 20 (20-20) | ||||||
| Rituximab plus CTC | ||||||||||
| First-line therapy, n# | 28 | 14 | 4 | 10 | 18/28 (64) | 11/13 (85) | 17/22 (77) | 3/28 (11) | 8/28 (29) | 3/28 (11) |
| CTC, mg/d, median dosage (range) | 60 (20-80) | 50 (20-80) | 40 (20-60) | 60 (50-70) | ||||||
| ≥ Second-line therapy, n** | 58 | 23 | 29 | 6 | 38/58 (66) | 15/17 (88) | 41/49 (84) | 3/58 (5) | 11/58 (19) | 5/58 (9) |
| CTC, mg/d, median dosage (range) | 40 (5-80) | 50 (10-80) | 40 (5-80) | 22.5 (15-40) | ||||||
| Rituximab plus alkylating agents regimen | ||||||||||
| First-line therapy, n†† | 12 | 1 | 0 | 11 | 10/12 (83) | 7/7 (100) | 8/10 (80) | 0/12 (0) | 5/12 (42) | 3/12 (25) |
| ≥ Second-line therapy, n‡‡ | 8 | 1 | 1 | 6 | 3/8 (38) | 2/5 (40) | 2/7 (29) | 3/8 (38) | 1/8 (13) | 1/8 (13) |
| Alternative therapy | ||||||||||
| First-line therapy, n | 11 | 5 | 4 | 2 | ||||||
| ≥ Second-line therapy, n | 25 | 9 | 12 | 4 | ||||||
CTC indicates corticosteroids.
Complete clinical response was defined as an improvement in all baseline clinical manifestations.
Renal response was defined by proteinuria < 0.5 g/d and/or the disappearance of hematuria and/or an improvement of GFR > 20% in case of baseline GFR < 70 mL/min/1.73m2.
Immunologic response was defined as a > 50% decrease in the baseline cryoglobulin level and/or a > 50% increase in the serum C4 fraction.
Refractory disease was defined as an improvement in less than one-half of the baseline clinical manifestations.
Including cyclophosphamide in 47 patients and chloraminophene in 8 patients.
Including cyclophosphamide in 25 patients and chloraminophene in 3 patients.
Including the administration of 375 mg/m2/wk for 4 weeks in 23 patients and of 1000 mg at day +1 and day +15 in 5 patients.
Including the administration of 375 mg/m2/ per week for 4 weeks in 49 patients and of 1000 mg at day 1 and day 15 in 9 patients.
Including rituximab/cyclophosphamide/prednisone in 9 patients and rituximab/cyclophosphamide/doxorubicin/vincristine/prednisone in 3 patients.
Including rituximab/cyclophosphamide/doxorubicin/vincristine/prednisone in 4 patients, rituximab/cyclophosphamide/prednisone in 3 patients, and rituximab/cyclophosphamide/fludarabine/prednisone in 1 patient.