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Therapeutic apheresis exchange in two patients with prolidase deficiency
1Department of Biochemistry A.Castellani, University of Pavia, Via Taramelli 3/B, I-27100 Pavia, Italy.
Background:
Prolidase deficiency is a rare genetic disorder for which a cure has not yet been found.
Objectives:
To assess the effectiveness of apheresis exchange as a new therapeutic approach.
Methods:
Apheresis exchanges were repeated monthly for four consecutive months, in parallel, on two patients, replacing prolidase-deficient red blood cells with normal filtered cells. Prolidase activity and urinary dipeptides were determined at regular intervals.
Results:
The constant presence of active prolidase inside cells allowed a continuous, although partial, degradation of imidodipeptides, with a concomitant improvement of skin ulceration.
Conclusions:
Apheresis exchange could be a reasonable way of obtaining a clinical improvement in these patients.