Get 20M+ Full-Text Papers For Less Than $1.50/day. Start a 14-Day Trial for You and Your Team.

Learn More →

Small Volume Plasmapheresis in the Management of Porphyria Cutanea Tarda

Small Volume Plasmapheresis in the Management of Porphyria Cutanea Tarda Abstract • A 49-year-old man with porphyria cutanea tarda was treated with repeated small volume plasmapheresis. After the circadian change of urinary porphyrins was found to be higher between midnight and noon, approximately 250 mL of plasma was removed at 8 AM and was replaced by physiological saline. Clinical disease manifestations and abnormal laboratory findings rapidly improved following a total of 20 plasmaphereses during a 24-week period. The blood hemoglobin and plasma total protein levels were unchanged. (Arch Dermatol 1983;119:752-755) References 1. Ippen H: Allgemein symptome der späten Hautporphyrie (Porphyria cutanea tarda) als Hinweise für deren Behandlung . Dtsch Med Wochenschr 1961;86:127-133.Crossref 2. Ramsay CA, Magnus IA, Turnbull A, et al: The treatment of porphyria cutanea tarda by venesection . Q J Med 1974;43:1-24. 3. Ippen H: Treatment of porphyria cutanea tarda by phlebotomy . Semin Hematol 1977;14:253-259. 4. Wechsler HL: Porphyria cutanea tarda: Report of a case treated with phlebotomies and supplemental iron . Arch Dermatol 1972;105:432-433.Crossref 5. Lundvall O: The effect of replenishment of iron stores after phlebotomy therapy in porphyria cutanea tarda . Acta Med Scand 1971;189:51-63.Crossref 6. Cripps DJ: Instrumentation and action spectra in light-associated diseases . J Invest Dermatol 1981;77:20-31.Crossref 7. Bickers DR: Treatment of the porphyrias: Mechanisms of action . J Invest Dermatol 1981;77:107-133.Crossref 8. Kushner JP, Lee GR, Nacht S: The role of iron in the pathogenesis of porphyria cutanea tarda: An in vitro model . J Clin Invest 1972;51:3044-3051.Crossref 9. Kushner JP, Steinmuller DP, Lee GR: The role of iron in the pathogenesis of porphyria cutanea tarda: II. Inhibition of uroporphyrinogen decarboxylase . J Clin Invest 1975;56:661-667.Crossref 10. Elder GH, Lee GB, Tovey JA: Decreased activity of hepatic uroporphyrinogen decarboxylase in sporadic porphyria cutanea tarda . N Engl J Med 1978;299:274-278.Crossref 11. Woo J, Treuting JJ, Cannon DC: Metabolic intermediates and inorganic ions , in Henry JB (ed): Clinical Diagnosis and Management by Laboratory Methods , ed 16. Philadelphia, WB Saunders Co, 1979, pp 294-298. 12. Auerbach R, Bystryn J-C: Plasmapheresis and immunosuppressive therapy: Effect on levels of intercellular antibodies in pemphigus vulgaris . Arch Dermatol 1979;115:728-730.Crossref 13. Isbister JP, Biggs JC, Penny R: Experience with large volume plasmapheresis in malignant paraproteinaemia and immune disorders . Aust NZ J Med 1978;8:154-164.Crossref 14. Jones JV, Cumming RH, Bacon PA, et al: Evidence for a therapeutic effect of plasmapheresis in patients with systemic lupus erythematosus . Q J Med 1979;48:555-576. 15. Allen BR, Parker S, Thompson GG, et al: The effect of treatment on plasma uroporphyrin levels in cutaneous hepatic porphyria . Br J Dermatol 1975;93:37-42.Crossref 16. Grossman ME, Bickers DR, Poh-Fitzpatrick MB, et al: Porphyria cutanea tarda: Clinical features and laboratory findings in 40 patients . Am J Med 1979;67:277-286.Crossref 17. Friedman BA, Schork MA, Mocniak JL, et al: Short-term and long-term effects of plasmapheresis on serum proteins and immunoglobulins . Transfusion 1975;15:467-472.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Dermatology American Medical Association

Small Volume Plasmapheresis in the Management of Porphyria Cutanea Tarda

Loading next page...
 
/lp/american-medical-association/small-volume-plasmapheresis-in-the-management-of-porphyria-cutanea-cYF1wRZuwa
Publisher
American Medical Association
Copyright
Copyright © 1983 American Medical Association. All Rights Reserved.
ISSN
0003-987X
eISSN
1538-3652
DOI
10.1001/archderm.1983.01650330044012
Publisher site
See Article on Publisher Site

Abstract

Abstract • A 49-year-old man with porphyria cutanea tarda was treated with repeated small volume plasmapheresis. After the circadian change of urinary porphyrins was found to be higher between midnight and noon, approximately 250 mL of plasma was removed at 8 AM and was replaced by physiological saline. Clinical disease manifestations and abnormal laboratory findings rapidly improved following a total of 20 plasmaphereses during a 24-week period. The blood hemoglobin and plasma total protein levels were unchanged. (Arch Dermatol 1983;119:752-755) References 1. Ippen H: Allgemein symptome der späten Hautporphyrie (Porphyria cutanea tarda) als Hinweise für deren Behandlung . Dtsch Med Wochenschr 1961;86:127-133.Crossref 2. Ramsay CA, Magnus IA, Turnbull A, et al: The treatment of porphyria cutanea tarda by venesection . Q J Med 1974;43:1-24. 3. Ippen H: Treatment of porphyria cutanea tarda by phlebotomy . Semin Hematol 1977;14:253-259. 4. Wechsler HL: Porphyria cutanea tarda: Report of a case treated with phlebotomies and supplemental iron . Arch Dermatol 1972;105:432-433.Crossref 5. Lundvall O: The effect of replenishment of iron stores after phlebotomy therapy in porphyria cutanea tarda . Acta Med Scand 1971;189:51-63.Crossref 6. Cripps DJ: Instrumentation and action spectra in light-associated diseases . J Invest Dermatol 1981;77:20-31.Crossref 7. Bickers DR: Treatment of the porphyrias: Mechanisms of action . J Invest Dermatol 1981;77:107-133.Crossref 8. Kushner JP, Lee GR, Nacht S: The role of iron in the pathogenesis of porphyria cutanea tarda: An in vitro model . J Clin Invest 1972;51:3044-3051.Crossref 9. Kushner JP, Steinmuller DP, Lee GR: The role of iron in the pathogenesis of porphyria cutanea tarda: II. Inhibition of uroporphyrinogen decarboxylase . J Clin Invest 1975;56:661-667.Crossref 10. Elder GH, Lee GB, Tovey JA: Decreased activity of hepatic uroporphyrinogen decarboxylase in sporadic porphyria cutanea tarda . N Engl J Med 1978;299:274-278.Crossref 11. Woo J, Treuting JJ, Cannon DC: Metabolic intermediates and inorganic ions , in Henry JB (ed): Clinical Diagnosis and Management by Laboratory Methods , ed 16. Philadelphia, WB Saunders Co, 1979, pp 294-298. 12. Auerbach R, Bystryn J-C: Plasmapheresis and immunosuppressive therapy: Effect on levels of intercellular antibodies in pemphigus vulgaris . Arch Dermatol 1979;115:728-730.Crossref 13. Isbister JP, Biggs JC, Penny R: Experience with large volume plasmapheresis in malignant paraproteinaemia and immune disorders . Aust NZ J Med 1978;8:154-164.Crossref 14. Jones JV, Cumming RH, Bacon PA, et al: Evidence for a therapeutic effect of plasmapheresis in patients with systemic lupus erythematosus . Q J Med 1979;48:555-576. 15. Allen BR, Parker S, Thompson GG, et al: The effect of treatment on plasma uroporphyrin levels in cutaneous hepatic porphyria . Br J Dermatol 1975;93:37-42.Crossref 16. Grossman ME, Bickers DR, Poh-Fitzpatrick MB, et al: Porphyria cutanea tarda: Clinical features and laboratory findings in 40 patients . Am J Med 1979;67:277-286.Crossref 17. Friedman BA, Schork MA, Mocniak JL, et al: Short-term and long-term effects of plasmapheresis on serum proteins and immunoglobulins . Transfusion 1975;15:467-472.Crossref

Journal

Archives of DermatologyAmerican Medical Association

Published: Sep 1, 1983

References