Therapeutic apheresis exchange in two patients with prolidase deficiency

✍ Scribed by A. Lupi; B. Casado; M. Soli; M. Bertazzoni; L. Annovazzi; S. Viglio; G. Cetta; P. Iadarola

• Book ID: 104460297
• Publisher: John Wiley and Sons
• Year: 2002
• Tongue: English
• Weight: 127 KB
• Volume: 147
• Category: Article
• ISSN: 0007-0963
• DOI: 10.1046/j.1365-2133.2002.04998.x

✦ Synopsis

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.