Acquired Fanconi syndrome is an indolent disorder in the absence of overt multiple myeloma.
Adult-acquired Fanconi syndrome (FS) is a rare complication of monoclonal gammopathy. We retrospectively reviewed 32 patients diagnosed with adult-acquired FS between April 1968 and June 2002 at Mayo Clinic (Rochester, MN). At diagnosis, most patients had monoclonal gammopathy of undetermined significance (MGUS) or smoldering multiple myeloma (SMM), with a median creatinine level of 176.8 microM (2.0 mg/dL; range, 79.56-327.08 microM [0.9-3.7 mg/dL]) and evidence of osteomalacia. During the average 65 months (range, 2-238 months) of follow-up, 5 patients developed end-stage renal disease (ESRD) and only 1 of 14 patients with MGUS transformed to multiple myeloma (MM). Also, 14 deaths occurred, with only 1 from ESRD but 4 from alkylator-related leukemia or myelodysplastic syndrome. Chemotherapy offered little benefit on renal functions of MGUS or SMM patients. In conclusion, FS associated with monoclonal gammopathy does not appear to confer an additional risk of subsequent evolution to MM. ESRD occurs late in the disease process.
Ma
Cynthia X
CX
Division of Hematology and Internal Medicine, Mayo Clinic, Rochester, MN 55905, USA.
Lacy
Martha Q
MQ
Rompala
John F
JF
Dispenzieri
Angela
A
Rajkumar
S Vincent
SV
Greipp
Philip R
PR
Fonseca
Rafael
R
Kyle
Robert A
RA
Gertz
Morie A
MA
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D003404:Creatinine / Q000378:metabolism; D018450:Disease Progression; D005198:Fanconi Syndrome / Q000150:complications / Q000453:epidemiology / Q000378:metabolism / Q000473:pathology*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009101:Multiple Myeloma / Q000150:complications / Q000453:epidemiology / Q000378:metabolism*; D010018:Osteomalacia / Q000378:metabolism; D010265:Paraproteinemias / Q000150:complications / Q000378:metabolism / Q000473:pathology; D051437:Renal Insufficiency / Q000453:epidemiology / Q000209:etiology; D012189:Retrospective Studies; D018570:Risk Assessment; D016019:Survival Analysis
D003404:Creatinine