Skip to main content

Table 2 Treatment for paraproteinemia

From: Paraproteinemic neuropathy: a practical review

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

 

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 1.5 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)

 

Pregnancy (category D)

Hypersensitivity to azathioprine

Rituximab

Alternative treatment for IgM-MGUS (off-label use)

Four weekly infusions of 375 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 0.2 mg/kg orally daily for 3 to 6 weeks

Hypersensitivity to chlorambucil

FDA pregnancy category D

Fludarabine

Alternative therapy for IgM-MGUS (off label use)

40 mg/m2 for 5 days every 28 days

Hypersensitivity to fludarabine

FDA pregnancy risk category D

Melphalan

Treatment for POEMS

For POEMS (high dose): 10 mg/m2 for 4 days every 28 days [58]

Hypersensitivity to melphalan

In combination with prednisone as treatment for AL

For AL: 0.15 mg/kg once daily for 7 days every 6 weeks, increasing the dose by 2 mg in each 6 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