Successful treatment of IgM paraproteinaemic neuropathy with fludarabine.
OBJECTIVE
To evaluate the response of four patients with IgM paraproteinaemic neuropathy to a novel therapy-pulsed intravenous fludarabine.


BACKGROUND
The peripheral neuropathy associated with IgM paraproteinaemia usually runs a chronic, slowly progressive course which may eventually cause severe disability. Treatment with conventional immunosuppressive regimens has been unsatisfactory. Fludarabine is a novel purine analogue which has recently been shown to be effective in low grade lymphoid malignancies.


METHODS
Four patients were treated with IgM paraproteinaemic neuropathy with intravenous pulses of fludarabine. Two of the four patients had antibodies to MAG and characteristic widely spaced myelin on nerve biopsy and a third had characteristic widely spaced myelin only. The fourth had an endoneurial lymphocytic infiltrate on nerve biopsy and a diagnosis of Waldenström's macroglobulinaemia.


RESULTS
In all cases subjective and objective clinical improvement occurred associated with a significant fall in the IgM paraprotein concentration in three cases. Neurophysiological parameters improved in the three patients examined. The treatment was well tolerated. All patients developed mild, reversible lymphopenia and 50% mild generalised myelosuppression, but there were no febrile episodes.


CONCLUSIONS
Fludarabine should be considered as a possible treatment for patients with IgM MGUS paraproteinaemic neuropathy.
Wilson
H C
HC
Department of Clinical Neuroscience, GKT School of Medicine, Guy's Hospital, London, UK.
Lunn
M P
MP
Schey
S
S
Hughes
R A
RA
D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D006801:Humans; D007075:Immunoglobulin M / Q000276:immunology*; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D010265:Paraproteinemias / Q000188:drug therapy* / Q000276:immunology; D010523:Peripheral Nervous System Diseases / Q000188:drug therapy* / Q000276:immunology; D014740:Vidarabine / Q000031:analogs & derivatives* / Q000627:therapeutic use
D007075:Immunoglobulin M; D007166:Immunosuppressive Agents; D014740:Vidarabine; C024352:fludarabine