Acquired Fanconi's syndrome associated with monoclonal gammopathies

Research output: Contribution to journalReview article

42 Citations (Scopus)

Abstract

Franconi's syndrome is a rare complication of the monoclonal gammopathies characterized by diffuse failure in reabsorption at the level of the proximal renal tubule resulting in glycosuria, generalized aminoaciduria, and hypophosphatemia. Current evidence suggests monoclonal light chains that are incompletely digested in renal tubule lysosomes cause renal injury. The light-chain fragments serve as a nidus for crystal formation, and the crystals interfere with a broad range of apical membrane transporters. Franconi's syndrome is predominantly associated with monoclonal κ in the urine, but rare instances of FS caused by λ light chains have been described. Overt hematologic malignancies such as multiple myeloma, Waldenstrom's macroglobulinemia, or other lymphoproliferative disorders occur in one third of patients. Clinical manifestations include slowly progressive renal failure and bone pain caused by osteomalacia. The osteomalacia is caused by chronic hypophosphatemia and may be exacerbated by secondary hyperparathyroidism and renal tubular acidosis. Treatment consists of supplementation with phosphorus, calcium, and vitamin D. The osteomalacia is often completely reversible with mineral supplementation. Patients with an associated symptomatic malignancy or rapidly progressive renal failure may also benefit from chemotherapy. The prognosis is good in the absence of overt malignant disease.

Original languageEnglish (US)
Pages (from-to)1273-1280
Number of pages8
JournalHematology/Oncology Clinics of North America
Volume13
Issue number6
DOIs
StatePublished - Jan 1 1999

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Fanconi Syndrome
Osteomalacia
Paraproteinemias
Hypophosphatemia
Light
Renal Insufficiency
Glycosuria
Renal Tubular Acidosis
Kidney
Waldenstrom Macroglobulinemia
Proximal Kidney Tubule
Secondary Hyperparathyroidism
Membrane Transport Proteins
Lymphoproliferative Disorders
Hematologic Neoplasms
Lysosomes
Multiple Myeloma
Vitamin D
Phosphorus
Minerals

ASJC Scopus subject areas

  • Hematology
  • Oncology

Cite this

Acquired Fanconi's syndrome associated with monoclonal gammopathies. / Lacy, Martha; Gertz, Morie.

In: Hematology/Oncology Clinics of North America, Vol. 13, No. 6, 01.01.1999, p. 1273-1280.

Research output: Contribution to journalReview article

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AB - Franconi's syndrome is a rare complication of the monoclonal gammopathies characterized by diffuse failure in reabsorption at the level of the proximal renal tubule resulting in glycosuria, generalized aminoaciduria, and hypophosphatemia. Current evidence suggests monoclonal light chains that are incompletely digested in renal tubule lysosomes cause renal injury. The light-chain fragments serve as a nidus for crystal formation, and the crystals interfere with a broad range of apical membrane transporters. Franconi's syndrome is predominantly associated with monoclonal κ in the urine, but rare instances of FS caused by λ light chains have been described. Overt hematologic malignancies such as multiple myeloma, Waldenstrom's macroglobulinemia, or other lymphoproliferative disorders occur in one third of patients. Clinical manifestations include slowly progressive renal failure and bone pain caused by osteomalacia. The osteomalacia is caused by chronic hypophosphatemia and may be exacerbated by secondary hyperparathyroidism and renal tubular acidosis. Treatment consists of supplementation with phosphorus, calcium, and vitamin D. The osteomalacia is often completely reversible with mineral supplementation. Patients with an associated symptomatic malignancy or rapidly progressive renal failure may also benefit from chemotherapy. The prognosis is good in the absence of overt malignant disease.

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