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Acquired Fanconi syndrome is an indolent disorder in the absence of overt multiple myeloma

Ma, Cynthia X. ; Lacy, Martha Q. ; Rompala, John F. ; Dispenzieri, Angela ; Rajkumar, S. Vincent ; Greipp, Philip R. ; Fonseca, Rafael ; Kyle, Robert A. ; Gertz, Morie A.

Blood, 2004-07, Vol.104 (1), p.40-42 [Periódico revisado por pares]

Washington, DC: Elsevier Inc

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Título:
Acquired Fanconi syndrome is an indolent disorder in the absence of overt multiple myeloma
Autor: Ma, Cynthia X. ; Lacy, Martha Q. ; Rompala, John F. ; Dispenzieri, Angela ; Rajkumar, S. Vincent ; Greipp, Philip R. ; Fonseca, Rafael ; Kyle, Robert A. ; Gertz, Morie A.
Assuntos: Adult ; Aged ; Aged, 80 and over ; Biological and medical sciences ; Chromosome fragility (bloom syndrome, ataxia telangiectasia, fanconi anemia, x-linked mental retardation...) ; Creatinine - metabolism ; Disease Progression ; Fanconi Syndrome - complications ; Fanconi Syndrome - epidemiology ; Fanconi Syndrome - metabolism ; Fanconi Syndrome - pathology ; Female ; Humans ; Immunodeficiencies. Immunoglobulinopathies ; Immunoglobulinopathies ; Immunopathology ; Male ; Medical genetics ; Medical sciences ; Middle Aged ; Multiple Myeloma - complications ; Multiple Myeloma - epidemiology ; Multiple Myeloma - metabolism ; Osteomalacia - metabolism ; Paraproteinemias - complications ; Paraproteinemias - metabolism ; Paraproteinemias - pathology ; Renal Insufficiency - epidemiology ; Renal Insufficiency - etiology ; Retrospective Studies ; Risk Assessment ; Survival Analysis
É parte de: Blood, 2004-07, Vol.104 (1), p.40-42
Notas: ObjectType-Article-1
SourceType-Scholarly Journals-1
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Descrição: 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 μM [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.
Editor: Washington, DC: Elsevier Inc
Idioma: Inglês

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Acquired Fanconi syndrome is an indolent disorder in the absence of overt multiple myeloma

Ma, Cynthia X. ; Lacy, Martha Q. ; Rompala, John F. ; Dispenzieri, Angela ; Rajkumar, S. Vincent ; Greipp, Philip R. ; Fonseca, Rafael ; Kyle, Robert A. ; Gertz, Morie A.

Washington, DC: Elsevier Inc

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