Therapeutic apheresis exchange in two patients with prolidase deficiency.

Abstract

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.

• Authors: A Lupi, B Casado, M Soli, M Bertazzoni, L Annovazzi, S Viglio, G Cetta, P Iadarola
• Journal: The British journal of dermatology (Br J Dermatol) Vol. 147 Issue 6 Pg. 1237-40 (Dec 2002) ISSN: 0007-0963 [Print] England
• PMID: 12452876 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Dipeptidases
• proline dipeptidase

Topics

• Adult
• Blood Component Removal (methods)
• Dipeptidases (deficiency)
• Electrophoresis, Capillary
• Erythrocytes (enzymology)
• Female
• Follow-Up Studies
• Humans
• Leg Ulcer (therapy)
• Male
• Middle Aged