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We present a case of distal renal tubular acidosis and acute renal insufficiency in a patient with Waldenström's macroglobulinemia. Distal renal tubular acidosis has been described in hypergammaglobulinemia, but not in patients with Waldenström's macroglobulinemia. To our knowledge, this is the first report to describe a possible association between distal renal tubular acidosis and Waldenström's macroglobulinemia.

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