Abstract
Background:
Polyneuropathy with IgM monoclonal gammopathy can be a disabling disorder necessitating treatment.
Methods:
In a prospective open label trial, 17 patients with disabling IgM MGUS polyneuropathy were treated with rituximab, a chimeric anti-CD-20 monoclonal antibody.
Results:
Rituximab induced an improvement of >or=1 point on the Overall Disability Sum Score in 2/17 patients, an improvement of >or=5% of the distal MRC sum score in 4/17 and the sensory sum score in 9/17 patients. Bone marrow investigations showed CD 20 B cell depletion in all patients. There were no serious adverse events. Compared with treatment with intermittent cyclophosphamide with prednisone or treatment with fludarabine, it shows a comparable response percentages but fewer side effects. The presence of anti-MAG and a disease duration shorter than 10 years may predict treatment response.
Conclusion:
Rituximab is a candidate for treatment of IgM MGUS polyneuropathy and should be further investigated in a double-blind randomised trial.
MeSH terms
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Age of Onset
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Anti-Inflammatory Agents / therapeutic use
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Antibodies, Monoclonal / adverse effects
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Antibodies, Monoclonal / therapeutic use*
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Antibodies, Monoclonal, Murine-Derived
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Antigens, CD20 / metabolism
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B-Lymphocytes / immunology
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Bone Marrow Cells / physiology
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Cyclophosphamide / therapeutic use
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Disability Evaluation
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Female
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Humans
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Immunoglobulin M / immunology*
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Immunologic Factors / adverse effects
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Immunologic Factors / therapeutic use*
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Immunosuppressive Agents / therapeutic use
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Lymphocyte Count
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Male
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Middle Aged
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Muscle Strength / physiology
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Neural Conduction / physiology
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Paraproteinemias / drug therapy*
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Paraproteinemias / immunology*
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Prednisone / therapeutic use
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Prospective Studies
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Rituximab
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Sensation / physiology
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Treatment Outcome
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Vidarabine / analogs & derivatives
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Vidarabine / therapeutic use
Substances
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Anti-Inflammatory Agents
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Antibodies, Monoclonal
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Antibodies, Monoclonal, Murine-Derived
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Antigens, CD20
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Immunoglobulin M
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Immunologic Factors
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Immunosuppressive Agents
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Rituximab
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Cyclophosphamide
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Vidarabine
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fludarabine
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Prednisone