We describe a 22-year-old Turkish woman with nephrotic syndrome who had a h
istory of acute myelocytic leukemia. After careful clinical evaluation, the
patient underwent a renal biopsy. Light microscopic examination showed dep
osition of Congo-positive material both In the mesangium and around the sma
ll vessels. By histochemical analyses, the deposited material was proved to
be amyloid A (AA). Because the patient's history did not reveal any event
that might explain the development of secondary amyloidosis, she was screen
ed for mutations causing familial Mediterranean fever (FMF) and was found t
o be homozygous for the M694V mutation by denaturing gradient gel electroph
oresis. We recommend that FMF-Phenotype If and the development of amyloid n
ephropathy, before or without other symptoms of FMF, should be kept In mind
in the face of unexplained proteinuria/amyloidosis, especially In high-ris
k ethnic groups. (C) 2001 by the National Kidney Foundation, Inc.