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stances this association is coincidental Nonetheless, when such cases are treated similarly to the IgM group with polyneuropathy, they respond as well or better Finally, it should be noted that in about 15 percent of our patients with anti-MAG antibody the illness has been mild and static for years at a time, even without treatment Only a small number of our patients have developed myeloma or Waldenstrom disease A bone marrow examination is generally not necessary unless the concentration of the paraprotein exceeds 3 g/dL or climbs progressively over years, or if other hematologic changes such as unexplained anemia or thrombocytopenia develop Treatment In most cases of uncomplicated monoclonal gammopathy, particularly if not of long-standing, plasma exchange produces transient improvement for several weeks to months, somewhat more so in patients with IgG and IgA types of neuropathy than in those with the IgM type (Dyck et al, 1991) The treatment regimen is approximately the same as for GBS, a total volume of approximately 200 to 250 mL/kg exchanged in each of 4 to 6 treatments over about 10 days and the removed plasma replaced with a mixture of albumin and saline In patients who are found to have serum activity against speci c components of myelin (particularly anti-MAG), plasma exchange alone has effected transient improvement in only 30 to 40 percent and sustained improvement in only 10 percent of our patients This discouraging outcome was obtained in cases with slowly progressive ataxia and marked distal sensory loss Sequential series of plasma exchanges every two to four months has sometimes resulted in repeated but transient responses According to some reports the response to immunosuppression with intravenous cyclophosphamide or udarabine, mycophenolate (Cellcept), or oral chlorambucil, when coupled with plasma exchanges has been somewhat better, at times allowing a reduction in the frequency of exchanges We have observed this only with cylcophosphamide (Gorson et al) Rituximab, which has the appeal of having a preferential effect on the B-cell lymphocyte population, is being tested in these diseases with mixed results so far Improvement with high-dose infused immunoglobulin (IVIG) has been effective in half of our cases with typical paraproteinemia, and 20 percent of those with anti-MAG neuropathy have improved for at least a brief period Other reports are not as positive In almost all instances immunosuppression and the plasma exchanges or IVIG must be repeated inde nitely at intervals of one to several months as determined by the clinical course An indwelling catheter is then usually required for venous access This group of neuropathies responds poorly or not at all to corticosteroids Osteosclerotic Myeloma (POEMS Syndrome) and Multiple Myeloma A neuropathy associated with multiple myeloma has already been mentioned under Paraneoplastic Neuropathy and Sensory Ganglionopathy This polyneuropathy complicates 13 to 14 percent of cases of multiple myeloma and has a disproportionately high association with the osteosclerotic form of the disease An abnormal monoclonal and excessive gamma globulin (mainly with the kappa light chain component in multiple myeloma but lambda in the osteosclerotic type) is found in the serum of over 80 percent of patients with myelomatous neuropathy In a special and small group of patients with single or other limited degrees of osteosclerotic myeloma, a sensorimotor polyneuropathy termed POEMS arises [Polyneuropathy (P) of moderate severity is associated with organomegaly (O), endocrinopathy (E), elevated M protein, and skin changes S (mainly hypertrichosis and skin thickening), hence the acronym POEMS] The same process has been referred to as the Crow-Fukase syndrome in Japan,. asp.net upc-a UPC-A . NET Control - UPC-A barcode generator with free . NET ...
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