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2017 ; 12
(1
): 150
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Fabry disease and incidence of cancer
#MMPMID28877708
Bird S
; Hadjimichael E
; Mehta A
; Ramaswami U
; Hughes D
Orphanet J Rare Dis
2017[Sep]; 12
(1
): 150
PMID28877708
show ga
BACKGROUND: Fabry disease is an X-linked lysosomal storage disorder caused by
deficient activity of ?-galactosidase A and the resulting accumulation of the
glycosphingolipid globotriaosylceramide (Gb3) and its derivatives, including
globotriaosylsphingosine (Lyso-Gb3). Increased cellular and plasma levels of Gb3
and Lyso-Gb3 affect multiple organs, with specific clinical consequences for the
kidneys, heart and brain. There is growing evidence that alterations in
glycosphingolipids may have an oncogenic role and this prompted a review of cases
of cancer and benign lesions in a large single centre cohort of Fabry patients.
We also explored whether there is a difference in the risk of cancer in Fabry
patients compared to the general population. RESULTS: Our results suggest that
Fabry patients may have a marginally reduced rate of all cancer (incidence rate
ratio 0.61, 95% confidence interval 0.37 to 0.99) but possibly increased rates of
melanoma, urological malignancies and meningiomas. CONCLUSION: Greater knowledge
and awareness of cancer in patients with Fabry disease may help identify at-risk
individuals and elucidate cancer mechanisms in this rare inherited disease, which
may potentially be relevant to the wider cancer population.
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