Patient (sex, age) | Disease course | Neuropathy duration* (years) | Haematological Disease | Pre-rituximab therapy | Rationale for rituximab | MRC† | INCAT† | Months before improvement | Duration of follow-up§ (years) |
1 (M, 72)9 | RR | 3 | Low-grade small B cell lymphoma | Ster, IVIG, AZA, Cyclosp | Refractory CIDP | 36–48 | 5-3 | 6 | 5 |
2 (F, 56) | RR | 2 | Ig M MGUS | Ster, IVIg, PE | To spare PE | 47–53‡ | 6-3 | 6 | 4 |
3 (M, 35) | RR | 10 | – | Ster, PE, IVIg, AZA, MM | To spare IVIg | 55–55 | 5-5 | – | 1 |
4 (M, 63) | RR | 0 | – | Ster, PE, IVIg, AZA | Refractory CIDP | 5–56 | 5-2 | 2 | 4 |
5 (F, 65) | CP | 4 | Ig A MGUS | Ster, PE, IVIg, AZA | Refractory CIDP | 56–47 | 3-4 | – | 1 |
6 (F, 67) | CP | 1 | Non-Hodgkin's lymphoma | Ster, PE, IVIg | Refractory CIDP | 46–54 | 5-3 | 1 | 2 |
7 (M, 59)10 | RR | 1 | ITP | Ster, IVIg | To spare IVIg, Ster | 47–60 | 5-0 | 1 | 2 |
8 (M, 50) | RR | 7 | Waldenstrom macroglobulinemia | Ster, IVIg | To spare IVIg | 47–51‡ | 5-3 | 1 | 2 |
9 (F, 30) | RR | 5 | – | Ster, IVIg, AZA | Refractory CIDP | 52–56 | 3-1 | 1 | 1 |
10 (M, 65) | RR | 4 | Ig M MGUS | Ster, IVIg, PE | Refractory CIDP | 48–52 | 6-4 | 2 | 1 |
11 (F, 59) | CP | 6 | Ig M MGUS | Ster, IVIg, AZA | Refractory CIDP | 40–50 | 8-4 | 4 | 1 |
12 (M, 53) | RR | 18 | – | Ster, IVIg, AZA, Cyclopho | To spare IVIg | 50–50 | 5-5 | – | 1 |
13 (M, 45) | CP | 19 | – | Ster, IVIg, AZA, MM, Cyclosp | To spare IVIg, Ster | 51–51 | 4-4 | – | 1 |
↵* From the onset of neurological symptoms.
↵† The first number refers to values at baseline and the second number to values at improvement.
↵‡ The degree of improvement obtained after PE and IVIg cycles and maintained after rituximab.
↵§ Which corresponded to duration of improvement in the responders.
AZA, azathioprine; CP, chronic-progressive; Cyclopho, cyclophosphamide; Cyclosp, ciyclosporin; INCAT, Inflammatory Neuropathy Cause and Treatment arm and leg disability scores; ITP, idiopathic thrombocytopenic purpura; IVIg, intravenous immunoglobulin; MGUS, monoclonal gammopathy of undetermined significance; MM, mycophenolate mofetil; MRC, Medical Research Council sum score; PE, plasma exchange; RR, relapsing-remitting; Ster, steroids.