Treatment | Indication | Dose | Major contraindications |
---|---|---|---|
Intravenous immune globulin (IVIG) | FDA-approved treatment for CIDP | 2 g/kg intravenously for 3–5 days, followed by maintenance dose of 1 g/kg every 3 weeks, followed by clinical reassessment | Hypersensitivity to IVIG |
FDA-approved treatment for multifocal motor neuropathy | 0.5-2.4 g/kg per month with clinical assessment followed by clinical reassessment | IgA deficiency | |
Treatment for IgA/IgG-MGUS-CIDP | Â | Immunoglobulin A deficiency | |
Alternative treatment for IgM-MGUS-distal demyelinating neuropathy (or rituximab, off-label use) | |||
Treatment of CANOMAD (off label use) | |||
Corticosteroids | First line treatment for CIDP associated with M protein (off-label use) | Prednisone: | Corticosteroid hypersensitivity |
An alternative treatment for IgM/A/G-MGUS (after rituximab or plasma exchange, off label use) | 1 to 1.5Â mg/kg/day orally | Fungal infection | |
Several treatment dosing regimens are available and duration is not agreed upon. | |||
Azathioprine | Alternative immunosuppressive treatment (after IVIG or corticosteroids) for CIDP associated with M protein (off-label use) | Â | Pregnancy (category D) |
Hypersensitivity to azathioprine | |||
Rituximab | Alternative treatment for IgM-MGUS (off-label use) | Four weekly infusions of 375Â mg/m2 rituximab [51] | Hypersensitivity to rituximab |
Alternative therapy for multifocal motor neuropathy (after IVIG, off label use) | |||
Chlorambucil | Alternative therapy for IgM-MGUS (off label use) | 0.1 to 0.2Â mg/kg orally daily for 3 to 6Â weeks | Hypersensitivity to chlorambucil |
FDA pregnancy category D | |||
Fludarabine | Alternative therapy for IgM-MGUS (off label use) | 40Â mg/m2 for 5Â days every 28Â days | Hypersensitivity to fludarabine |
FDA pregnancy risk category D | |||
Melphalan | Treatment for POEMS | For POEMS (high dose): 10Â mg/m2 for 4Â days every 28Â days [58] | Hypersensitivity to melphalan |
In combination with prednisone as treatment for AL | For AL: 0.15Â mg/kg once daily for 7Â days every 6Â weeks, increasing the dose by 2Â mg in each 6Â week course. Should be used in combination with prednisone. | Should not be used in patients whose disease has demonstrated prior melphalan resistance | |
Plasmapheresis (plasma exchange) | Alternative treatment of IgG/A MGUS | 3 to 5 exchanges every other day | Risks associated with frequent vascular access |
May be helpful in severe cases of cryoglobulinemia. | Risk of transmission of infective agents if fresh plasma is used as replacement fluid | ||
Anaphylaxis and allergic reactions may occur with reinfusion of plasma substitute | |||
Autologous peripheral stem cell transplant | First line treatment of POEMS syndrome | Myeloablative doses of chemotherapy and/or radiation therapy followed by infusion of peripheral blood stem cells | Patients who undergo HCT are at risk for bacterial, viral, and fungal infections |
Alternative treatment of AL, used in combination with melphalan | Early adverse effects include: nausea, vomiting, diarrhea, mouth sores | ||
Later adverse effects include: cataracts, sterility, increased risk of other neoplasias | |||
Radiation therapy | First line treatment of dominant sclerotic plasmacytoma in POEMS syndrome | Radiation therapy delivered to osteosclerotic lesions in doses of 40–50 Gy | Adverse effects depend upon the location of the area irradiated |
Increased risk of developing second malignancies in patients with Hodgkin’s Lymphoma |