In a recent issue of this journal, Simmons and associates described 5 cases of polyneuropathy associated with IgA monoclonal gammopathy of undetermined significance (MGUS) and reviewed 6 similar cases from the literature.' These authors do not mention the setting of nonmalignant IgA gammopathies associated with peripheral neuropathy and systemic signs, such as papilledema, thrombocytosis, and multicentric Castleman's disease. 1--3,5,6,8 In these patients, systemic manifestations are those of the POEMS (Polyneuroputhy, Orgunomegaly, Endocrinoputhy, M protein, Skin changes) ~y n d r o m e ,~ but malignant B-cell proliferation, including osteosclerotic or osteolytic myeloma, extramedullary plasmocytoma, and lymphoma, cannot be detected by appropriate investigations. We describe a patient with IgAh monoclonal gammopathy, polyneuropathy, systemic symptoms, and multicentric Castleman's disease without evidence of immunocytic malignancy.A 68-year-old man came to medical attention because of severe arteriopathy in the lower limbs necessitating bilateral aortofemoral bypass. Subsequently, chronic diarrhea, weight loss (32 kg), and a sensorimotor polyneuropathy with generalized tendinous areflexia developed. Extraneurologic symptoms included moderate skin thickening and hirsutism, finger clubbing, white nails, facial telangectasias, and pitting edema. Hepatosplenomegaly and diffuse lymphadenopathy were noted. CSF contained 150 mg/dL protein and no cell. Nerve conduction velocities were initially normal and subsequently dropped in the range of demyelinating disorders. Nerve biopsy showed marked myelinated fiber loss, endoneurial edema, and perivascular mononuclear cuffs. Endocrine screenings showed an increasing thyroid dysfunction with low free T,, normal free T,, and increased TSH, low serum testosterone, normal serum oestradiol, high LH, and normal FSH.
