Marked hyperamylasaemia associated with an amylase-producing multiple myeloma appears to be a fairly unusual phenomenon. The present report describes a fatal case of multiple myeloma associated with paraneoplastic hyperamylasaemia without evidence of pancreatic or salivary gland involvement. Serum and urine amylase levels paralleled the myeloma response to chemotherapy and disease progression. The importance of paraneoplastic hyperamylasaemia as a useful myeloma marker to monitor disease progression and treatment response is emphasized. Conventional chemotherapy at diagnosis and salvage treatment with bortezomib at relapse failed to achieve a long-term response and to decrease the serum amylase to a normal level.

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