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Transfusion Therapy in a Patient With Erythropoietic Protoporphyria

Transfusion Therapy in a Patient With Erythropoietic Protoporphyria Abstract • An 8-year-old boy with erythropoietic protoporphyria had a marked decrease in photosensitivity symptoms after several transfusions with washed, packed RBCs. The reduction in photosensitivity was associated with a decline in free erythrocyte protoporphyrin levels. (Arch Dermatol 117:99-101, 1981) References 1. Harber LC: Porphyria , in Demis DJ, Dobson RL, McGuire J (eds): Clinical Dermatology . Hagerstown, Md, Harper & Row Publishers Inc, 1976, vol 2, pp 13-17. 2. DeLeo VA, Poh-Fitzpatrick M, Mathews-Roth M, et al: Erythropoietic protoporphyria . Am J Med 1976;60:8-22.Crossref 3. Bottomley SS, Tanaka M, Everett MA: Diminished bone marrow ferrochelatase activity in protoporphyria . Clin Res 1974;22:384a. 4. Mathews-Roth MM, Pathak MA, Fitzpatrick TB, et al: Beta carotene therapy for erythropoietic protoporphyria and other photosensitivity diseases . Arch Dermatol 1977;113:1229-1232.Crossref 5. Moshell A, Bjornson L: Protoporphyria: Mechanism of photoprotection by beta-carotene . J Invest Dermatol 1977;68:157-160.Crossref 6. Scholnick P, Marven HS, Schmid R: Erythropoietic protoporphyria: Evidence for multiple sites of excess protoporphyrin formation . J Clin Invest 1971;50:203-207.Crossref 7. Piomelli S, Lamola AA, Poh-Fitzpatrick MB, et al: Erythropoietic protoporphyria and lead intoxication: The molecular basis for difference in cutaneous photosensitivity . J Clin Invest 1971;56:1519-1527.Crossref 8. Russell MO, Goldberg HI, Reis L, et al: Transfusion therapy for cerebrovascular abnormalities in sickle cell disease . J Pediatr 1976;88:382-387.Crossref 9. Beard MEJ, Necheles TF, Allen DM: Intensive transfusion therapy in thalassemia major . Pediatrics 1967;40:912-913. 10. Miao LL, Mathews-Roth MM, Poh-Fitzpatrick MB: Beta carotene treatment and erythrocytic protoporphyrin levels . Arch Dermatol 1979;115:818.Crossref 11. Giblett ER: Blood groups and blood transfusion , in Isselbacher KJ, Adams RD, Braunwald E (eds): Harrison's Principles of Internal Medicine , ed 9. New York, McGraw-Hill Book Co, 1975, pp 1575-1576. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Dermatology American Medical Association

Transfusion Therapy in a Patient With Erythropoietic Protoporphyria

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References (15)

Publisher
American Medical Association
Copyright
Copyright © 1981 American Medical Association. All Rights Reserved.
ISSN
0003-987X
eISSN
1538-3652
DOI
10.1001/archderm.1981.01650020041022
Publisher site
See Article on Publisher Site

Abstract

Abstract • An 8-year-old boy with erythropoietic protoporphyria had a marked decrease in photosensitivity symptoms after several transfusions with washed, packed RBCs. The reduction in photosensitivity was associated with a decline in free erythrocyte protoporphyrin levels. (Arch Dermatol 117:99-101, 1981) References 1. Harber LC: Porphyria , in Demis DJ, Dobson RL, McGuire J (eds): Clinical Dermatology . Hagerstown, Md, Harper & Row Publishers Inc, 1976, vol 2, pp 13-17. 2. DeLeo VA, Poh-Fitzpatrick M, Mathews-Roth M, et al: Erythropoietic protoporphyria . Am J Med 1976;60:8-22.Crossref 3. Bottomley SS, Tanaka M, Everett MA: Diminished bone marrow ferrochelatase activity in protoporphyria . Clin Res 1974;22:384a. 4. Mathews-Roth MM, Pathak MA, Fitzpatrick TB, et al: Beta carotene therapy for erythropoietic protoporphyria and other photosensitivity diseases . Arch Dermatol 1977;113:1229-1232.Crossref 5. Moshell A, Bjornson L: Protoporphyria: Mechanism of photoprotection by beta-carotene . J Invest Dermatol 1977;68:157-160.Crossref 6. Scholnick P, Marven HS, Schmid R: Erythropoietic protoporphyria: Evidence for multiple sites of excess protoporphyrin formation . J Clin Invest 1971;50:203-207.Crossref 7. Piomelli S, Lamola AA, Poh-Fitzpatrick MB, et al: Erythropoietic protoporphyria and lead intoxication: The molecular basis for difference in cutaneous photosensitivity . J Clin Invest 1971;56:1519-1527.Crossref 8. Russell MO, Goldberg HI, Reis L, et al: Transfusion therapy for cerebrovascular abnormalities in sickle cell disease . J Pediatr 1976;88:382-387.Crossref 9. Beard MEJ, Necheles TF, Allen DM: Intensive transfusion therapy in thalassemia major . Pediatrics 1967;40:912-913. 10. Miao LL, Mathews-Roth MM, Poh-Fitzpatrick MB: Beta carotene treatment and erythrocytic protoporphyrin levels . Arch Dermatol 1979;115:818.Crossref 11. Giblett ER: Blood groups and blood transfusion , in Isselbacher KJ, Adams RD, Braunwald E (eds): Harrison's Principles of Internal Medicine , ed 9. New York, McGraw-Hill Book Co, 1975, pp 1575-1576.

Journal

Archives of Dermatology – American Medical Association

Published: Feb 1, 1981

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