Fanconi syndrome in lymphoma patients: report of the first case series.
BACKGROUND
Fanconi syndrome (FS) is a generalized transport defect in the proximal renal tubule leading to renal losses of phosphate, calcium, uric acid, bicarbonates as well as glucose, amino acids and other organic compounds. It is caused by inherited or acquired disorders including low mass or high mass multiple myeloma.


OBJECTIVE
To report the first case series of patients with lymphoma and FS.


METHODS
Patients with lymphoma and FS were identified in the nephrology department of two teaching hospitals in Paris, France and Ghent, Belgium. FS was defined by the presence of at least three out of the four following criteria: hypophosphataemia, metabolic acidosis, normoglycaemic glucosuria and hypokalaemia. Patients files were reviewed and relevant data were collected.


RESULTS
Eight patients with lymphoma and FS were identified. In six patients, the lymphoma was of the acute T cell leukaemia/lymphoma (ATLL) type, related to human T cell lymphotropic virus 1 (HTLV1) infection. In all patients, FS was severe requiring supplementation. A kidney biopsy performed in a patient with post-transplantation primary renal lymphoma disclosed intense proximal tubule infiltration by lymphomatous cells. In one patient with ATLL, FS features regressed following the successful treatment of lymphoma.


CONCLUSIONS
Patients with lymphoma require careful monitoring for features of FS; lymphoma should also be added to the spectrum of disorders associated to FS. Prospective studies are needed to ascertain the implication of HTLV1 in the genesis of FS.
Nephrology Department, Universite Paris Descartes, AP-HP, Hopital Necker, Paris, France.
Jill
J
Vanmassenhove
Marion
M
Sallée
Philippe
P
Guilpain
Raymond
R
Vanholder
Alexandra
A
De Potter
Louis
L
Libbrecht
Felipe
F
Suarez
Olivier
O
Hermine
Fadi
F
Fakhouri
D000328:Adult; D000368:Aged; D001530:Belgium; D001706:Biopsy; D015897:Comorbidity; D005198:Fanconi Syndrome / Q000175:diagnosis / Q000453:epidemiology* / Q000821:virology; D005260:Female; D005602:France; D015368:Human T-lymphotropic virus 1 / Q000502:physiology; D006801:Humans; D007668:Kidney / Q000473:pathology; D008223:Lymphoma / Q000175:diagnosis / Q000453:epidemiology*; D008297:Male; D008875:Middle Aged; D054218:Precursor T-Cell Lymphoblastic Leukemia-Lymphoma / Q000175:diagnosis / Q000453:epidemiology*