Table 4.
Flare up effect.
| Patient | 1 | 2 | 3 | 4 | 5 | ALL/median |
|---|---|---|---|---|---|---|
| PP/CT cohort | CT | CT | CT | CT | CT | 100% CT |
| Sex M/F | F | M | M | F | F | 2/3 |
| Age at Rx initiation (years) | 67 | 50 | 75 | 67 | 53 | 67 |
| Type of cryoglobulinemia | I | I | II | I | II | 3/5 I 2/5 II |
| Etiology | BCL + AID | BCL | BCL | MGUS | MGUS | 100% BCL 1/5 AI |
| Concomitant treatment | CS + ChT | / | CS | / | / | |
| Initial organ involvement | Pulmonary | Digital ischemia | Renal Cutaneous Neuropathy |
Cutaneous necrosis | Cutaneous Arthritis |
|
| Flare manifestations | Pulmonary with AH suspicion | Digital ischemia | Renal Cutaneous necrosis and legs ischemia |
Cutaneous necrosis | Cutaneous; Arthritis |
|
| Biological markers | ||||||
| C3 (mg/L) | 0,42 | na | 0,58 | na | na | |
| C4 (mg/L) | 0,02 | na | 0,03 | na | na | |
| IF | IgM kappa | IgM kappa | IgM kappa | IgG lambda | IgM kappa | |
| RF (UI/mL) | <10 | <10 | 125 | na | na | |
| ICU admission | Yes | No | Yes | No | na | 2/5 |
| Flare-up management | 0 | PEx | CS + PEx | PEx | endoxan | 3/5 PEx |
| TTNT | NR | 2 months | 1 months | 2 months | 2 months | |
| Death related to CV flare | Yes | No | Yes | Yes | No | 3/5 |
| OS | 1 months | NR | 4 months | 39 months | NR | |
Flare-up effect manifestations and outcomes for the five patients concerned in the CT cohort. BCL: B cell lymphoproliferation; AID: auto-immune disease; CS: corticosteroids; ChT: chemotherapy; na: not available; PP: plasmapheresis; OS: overall survival; TTNT: time to next treatment; NR: not reached.