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TONI DEBRE FANCONI SYNDROME DURING MYELOMA, ABOUT 8 CASES
Author(s): ,
Rahali Cherif
Affiliations:
Hematology,Central Hospital Mohammed Seghir Nekkache, Algiers, Algeria,Algiers,Algeria
Belakehal Salah Eddine
Affiliations:
Hematology,Central Hospital Mohammed Seghir Nekkache, Algiers, Algeria,Algiers,Algeria
(Abstract release date: 05/18/17) EHA Library. Rahali C. 05/18/17; 182732; PB2018
Cherif Rahali
Cherif Rahali
Contributions
Abstract

Abstract: PB2018

Type: Publication Only

Background
The cast nephropathy with cylinders is the most frequent renal complication of the myeloma, which results from a catabolism of the light chains by the tubular cells and can lead a tubular chronic suffering showing itself by a syndrome of acquired Toni-Debré-Fanconi marked by a glycosuria, a phosphaturia, an aminoaciduria, a sometimes severe and sometimes revealing hypokalemia.

Aims
We reporting some observations informed by Multiple Myeloma complicated with a Fanconi syndrome.

Methods
From January 2000 till December 2010: 78 cases of Multiple Myeloma were brought together, whose circumstance of discovery 22 cases with renal failure, it’s was a evolutes complications in 12 cases; and in 10 cases it’s discovered at diagnosis. The renal achievement is dominated by Tubule disease in 11 cases, Randall syndrome 8 cases, and Nephrotic syndrome in 3 cases. The tubule disease of Fanconi is suspected at only 8 patients: in front of the presence of a renal glycosuria (without associated diabetes) and a frank proteinuria in the majority of the cases, with a hypophosphatemia and a fickle hypokalemia.

Results
The clinico-epidemiological and immuno-biological characters of these 8 patients are the following ones:

- The median age is of 64 years (39-76), sex ratio=3. The osseous pains and the muscular cramps dominate the clinical presentation with constant diffuse demineralization in the radiology.
- The patients were classified (according to the Salmon-Durie classification): IIIB (3 cases) and IIB (5 cases). ISS 3 in majority of the cases.
- The monoclonal immunoglobulin observed: IgG kappa: 4cases, IgA kappa: 2cases, light chain kappa: 2cases. With a Bence Jones proteinuria isotype kappa and a glycosuria in the majority of the cases.
- The gravity of the renal faillure, based on the clearance of the creatinine: with an average clearance of 16,19 ml/min (4-37): several in 5cases, terminal in 3cases.
- We note more of hypocalcaemia while the hypercalcaemia is noted in a single case, the hypophosphatemia is found in half of the cases.
The therapeutics is double:
- Symptomatic: alkaline hydration, correction of the metabolic disorders and sometimes the renal extra purge (indicated in 3cases).
- Specific: chemotherapies VAD 7cases, a patient died by cardio-vascular complication. Under treatment the recovery of the renal function is obtained in 3 cases, to the rests of the patients persists a stable renal failure.

Conclusion
The Syndrome of Fanconi is a frequent and often formidable complication during Myeloma, observed to 30-40% of the patients in an autopsique series. It is necessary to think to it in front of any renal achievement in myeloma of kappa light chain with renal glycosuria, a generalized amino-aciduria and a hypophosphatemia resulting respectively from a defect of the transport of the glucose, from amino acids and from phosphates by the renal proximal tubule. To improve the osseous and renal appearances, it is necessary to realize a calcic supplementation, phosphorous and by the vitamin D active, as well as the correction of the acidose and a specific treatment reducing the excretion renal of the light chains.

Session topic: 14. Myeloma and other monoclonal gammopathies - Clinical

Keyword(s): Multiple Myeloma

Abstract: PB2018

Type: Publication Only

Background
The cast nephropathy with cylinders is the most frequent renal complication of the myeloma, which results from a catabolism of the light chains by the tubular cells and can lead a tubular chronic suffering showing itself by a syndrome of acquired Toni-Debré-Fanconi marked by a glycosuria, a phosphaturia, an aminoaciduria, a sometimes severe and sometimes revealing hypokalemia.

Aims
We reporting some observations informed by Multiple Myeloma complicated with a Fanconi syndrome.

Methods
From January 2000 till December 2010: 78 cases of Multiple Myeloma were brought together, whose circumstance of discovery 22 cases with renal failure, it’s was a evolutes complications in 12 cases; and in 10 cases it’s discovered at diagnosis. The renal achievement is dominated by Tubule disease in 11 cases, Randall syndrome 8 cases, and Nephrotic syndrome in 3 cases. The tubule disease of Fanconi is suspected at only 8 patients: in front of the presence of a renal glycosuria (without associated diabetes) and a frank proteinuria in the majority of the cases, with a hypophosphatemia and a fickle hypokalemia.

Results
The clinico-epidemiological and immuno-biological characters of these 8 patients are the following ones:

- The median age is of 64 years (39-76), sex ratio=3. The osseous pains and the muscular cramps dominate the clinical presentation with constant diffuse demineralization in the radiology.
- The patients were classified (according to the Salmon-Durie classification): IIIB (3 cases) and IIB (5 cases). ISS 3 in majority of the cases.
- The monoclonal immunoglobulin observed: IgG kappa: 4cases, IgA kappa: 2cases, light chain kappa: 2cases. With a Bence Jones proteinuria isotype kappa and a glycosuria in the majority of the cases.
- The gravity of the renal faillure, based on the clearance of the creatinine: with an average clearance of 16,19 ml/min (4-37): several in 5cases, terminal in 3cases.
- We note more of hypocalcaemia while the hypercalcaemia is noted in a single case, the hypophosphatemia is found in half of the cases.
The therapeutics is double:
- Symptomatic: alkaline hydration, correction of the metabolic disorders and sometimes the renal extra purge (indicated in 3cases).
- Specific: chemotherapies VAD 7cases, a patient died by cardio-vascular complication. Under treatment the recovery of the renal function is obtained in 3 cases, to the rests of the patients persists a stable renal failure.

Conclusion
The Syndrome of Fanconi is a frequent and often formidable complication during Myeloma, observed to 30-40% of the patients in an autopsique series. It is necessary to think to it in front of any renal achievement in myeloma of kappa light chain with renal glycosuria, a generalized amino-aciduria and a hypophosphatemia resulting respectively from a defect of the transport of the glucose, from amino acids and from phosphates by the renal proximal tubule. To improve the osseous and renal appearances, it is necessary to realize a calcic supplementation, phosphorous and by the vitamin D active, as well as the correction of the acidose and a specific treatment reducing the excretion renal of the light chains.

Session topic: 14. Myeloma and other monoclonal gammopathies - Clinical

Keyword(s): Multiple Myeloma

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