Abstract
Peripheral neuropathy is seen in 15% of patients with IgA monoclonal gammopathy. Treatment and prognosis of dysproteinemic neuropathy is usually guided by the underlying plasma cell disorders, which could be either benign or malignant. The true incidence of hematologic malignancy in patients with neuropathy associated with IgA monoclonal gammopathy is not known. However, patients with IgA M-protein are generally at increased risk for malignant transformation. Since neuropathy may be the first and only organ involvement, neurologists are key contributors in identifying each patient's plasma cell dyscrasia. We report two patients who presented with severe progressive polyneuropathy, had a detectable low-level IgA lambda paraproteinemia dismissed as incidental. Both were diagnosed later with a combination of malignant plasma cell dyscrasia and AL amyloidosis resulting in multiorgan failure and death. Both patients demonstrated red flags for malignant progression including abnormal serum free light chain, rapidly progressive debilitating neuropathy refractory to immunotherapy, prominent autonomic dysfunction, and weight loss. In summary, patients with IgA monoclonal gammopathy presenting with polyneuropathy can be at risk for malignant transformation. Failure to investigate for hematologic malignancy and AL amyloidosis may cause significant delays in treatment and result in fatal outcomes.