Therapeutic apheresis exchange in two patients with prolidase deficiency.
BACKGROUND
Prolidase deficiency is a rare genetic disorder for which a cure has not yet been found.


OBJECTIVE
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.
Lupi
A
A
Department of Biochemistry A.Castellani, University of Pavia, Via Taramelli 3/B, I-27100 Pavia, Italy.
Casado
B
B
Soli
M
M
Bertazzoni
M
M
Annovazzi
L
L
Viglio
S
S
Cetta
G
G
Iadarola
P
P
D000328:Adult; D001781:Blood Component Removal / Q000379:methods*; D004150:Dipeptidases / Q000172:deficiency*; D019075:Electrophoresis, Capillary; D004912:Erythrocytes / Q000201:enzymology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007871:Leg Ulcer / Q000628:therapy*; D008297:Male; D008875:Middle Aged
D004150:Dipeptidases; C022449:proline dipeptidase