Therapeutic apheresis exchange in two patients with prolidase deficiency

Br J Dermatol. 2002 Dec;147(6):1237-40. doi: 10.1046/j.1365-2133.2002.04998.x.

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.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

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

Substances

  • Dipeptidases
  • proline dipeptidase