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Sydney 2005: Cryoglobulinaemia, POEMS and Gammopathy Associated Neuropathy
By Morie Gertz, MD
04.12.05
Dr. Gertz gave a presentation on monoclonal gammopathies other than MM, MGUS, and AL amyloidosis—cryoglobulinemia and POEMS (osteosclerotic myeloma). At the Mayo clinic (Rochester, Minnesota, United States), 139 out of 700 “other” patients were diagnosed with osteosclerotic myeloma; 227 out of 700 were diagnosed with cryoglobulinemia. The median age of patients with cryoglobulinemia is 58; presenting with symptoms including purpura, renal impairment, edema, peripheral neuropathy, and arthralgia. Underlying disorders include hepatitis C virus (HCV), lymphoplasmacytic disorder (LPD), and autoimmune disease. The most common clinical manifestation with LPD is skin ulceration; whereas neuropathy and Raynaud’s syndrome are the more common autoimmune diseases. Treatment options include plasmapheresis, interferon, immunoglobulin infusion, and recent use of rituximab. Response rates, regardless of type of treatment, are 60% to 70% and the only predictor of survival is age. POEMS is associated with monoclonal protein of the lambda type. The majority of patients present with sclerotic bone lesions and 11% present with Castleman’s disease. The median age of patients is 51 years with the following symptoms: neuropathy, organomegaly, M-protein spike, and thrombocytopenia. This disease is non-fatal and the final outcome is patients becoming wheelchair bound. Treatment options include radiation, melphalan-based chemotherapy, or combination chemotherapy with alkylating agents. More recently, a study using PBSCT was found to be effective in patients who have failed radiotherapy with results of significantly improved neural impairment scores.


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