Figure 3.

INCAT-ODSS before and after first (n = 48), second (n = 18), and third therapy (n = 6) with CYP, RTX, and/or BTZ for patients refractory to first-line treatment. Mean duration until first treatment with CYP, RTX, and/or BTZ was 39.7 months, until second treatment 67.1 months, and until third treatment 119.8 months. For comparison, INCAT-ODSS at 40, 67, and 120 months after initial diagnosis of patients not treated with CYP, RTX, and/or is shown in the figure (remaining patients). At 12 months before first therapy, the INCAT-ODSS did not differ significantly between both groups but was higher in patients treated with CYP, RTX, and/or BTZ (3.0 versus 2.3, t test, p = 0.09). At the time of first treatment with CYP, RTX, and/or BTZ, the score increased to 4.4 points in patients treated with CYP, RTX, and/or BTZ and stayed at 2.4 points in the remaining patients (t test, p < 0.01). At 12 months after first therapy with CYP, RTX, and/or BTZ, the INCAT-ODSS decreased, showing the therapeutic response. However, patients treated with CYP, RT,X and/or BTZ were still more disabled than the remaining patients (INCAT-ODSS 4.0 versus 2.2 at 12 months after therapy; p < 0.01). Similar results were found for second and third therapy. The INCAT-ODSS in patients receiving a third therapy increased to up to 6.5 points, whereas the remaining patients stayed between 2.5 and 3.5 points (p < 0.01).

*p = 0.05, **p = 0.01,***p = 0.001,****p < 0.001.

BTZ, bortezomib; CYP, cyclophosphamide; INCAT-ODSS, inflammatory neuropathy cause and treatment overall disability score; RTX, rituximab.