TY - JOUR
T1 - Acquired Fanconi syndrome is an indolent disorder in the absence of overt multiple myeloma
AU - Ma, Cynthia X.
AU - Lacy, Martha Q.
AU - Rompala, John F.
AU - Dispenzieri, Angela
AU - Rajkumar, S. Vincent
AU - Greipp, Philip R.
AU - Fonseca, Rafael
AU - Kyle, Robert A.
AU - Gertz, Morie A.
PY - 2004/7/1
Y1 - 2004/7/1
N2 - 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 μM (2.0 mg/dL; range, 79.56-327.08 μLM [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.
AB - 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 μM (2.0 mg/dL; range, 79.56-327.08 μLM [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.
UR - http://www.scopus.com/inward/record.url?scp=3042756207&partnerID=8YFLogxK
U2 - 10.1182/blood-2003-10-3400
DO - 10.1182/blood-2003-10-3400
M3 - Article
C2 - 15010372
AN - SCOPUS:3042756207
SN - 0006-4971
VL - 104
SP - 40
EP - 42
JO - Blood
JF - Blood
IS - 1
ER -