Patients (particularly elderly) undergoing evaluation for peripheral neuropathy of unknown cause should be screened for the presence of a monoclonal protein (M protein). The association of a neuropathy and a paraproteinaemia such as Waldenstrom's Macroglobulinaemia (WM) is not uncommon with the former antedating the haematologic symptoms by several years. Response to treatment has varied from good to very poor. We describe a case of WM presenting as a subacute demyelinating peripheral neuropathy. There was prompt resolution of the neuropathy with intravenous immunoglobulin therapy. Subsequent treatment with cyclophosphamide and plasmapheresis resulted in complete clinical remission with no further neurological relapses.
A case of Waldenstrom's macroglobulinemia with classical findings of IgM paraproteinaemia and a typical lymphoplasmacytic marrow infiltrate is reported and the treatment of this patient outlined.
Irreversible optic nerve dysfunction associated with central retinal vein occlusion (CRVO) is an unusual but important complication of Waldenstrom Macroglobulinemia (WM). Acute visual loss in CRVO is mainly due the severe macular oedema. However, ischaemic optic neuropathy needs to be considered in patients with CRVO when, (i) there is a relative afferent papillary defect and central scotoma, (ii) the visual acuity is not consistent with the retinal pathology, and (iii) the visual defects persisted despite resolution of macular oedema following treatment of the hyperviscosity state. The ischaemic type of CRVO is associated with a poor visual prognosis and the presenting visual acuity has a prognostic role. We report the first description of irreversible unilateral optic nerve damage associated with CRVO in a patient with WM.