Description

Intracellular deposits associated with drug-induced phospholipidosis may resemble those seen in Fabry disease. 


Clinical features:

(1) The patient has a history of chloroquine therapy.

(2) The patient develops a low-grade nephropathy with proteinuria, hematuria and a decline in renal function.

(3) The renal dysfunction reverses after discontinuation of chloroquine therapy.

 

Laboratory findings:

(1) A renal biopsy shows intracellular accumulation of phospholipids in glomerular cells, resulting in foam cells. On electron microscopy these lysosomal deposits consist of alternating dense and pale lipid material, termed myeline figures ("zebra bodies").

(2) The plasma alpha-galactosidase A activity is normal.

(3) Sequencing of the alpha-galactosidase A gene does not show a mutation.


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