Sideroblastic Anemia With Markedly Increased Free Erythrocyte Protoporphyrin Without Dermal Photosensitivity

A patient with idiopathic sideroblastic anemia and atypical clinical and biochemical findings is described. He had a greatly increased erythrocyte and plasma protoporphyrin, but normal urine and fecal porphyrins. The erythrocyte protoporphyrin had a fluorescence spectrum typical of free protoporphyr...

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Bibliographic Details
Published in:Blood 1982-03, Vol.59 (3), p.628-633
Main Authors: Romslo, Inge, Brun, Atle, Sandberg, Sverre, Bottomley, Sylvia S., Høvding, Gunnar, Talstad, Ingebrigt
Format: Article
Language:English
Subjects:
Aged
Anemia, Sideroblastic - complications
Anemia, Sideroblastic - diagnosis
Bone Marrow - ultrastructure
Erythrocytes - analysis
Feces - analysis
Ferrochelatase - metabolism
Hemolysis
Humans
Iron
Male
Mitochondria - ultrastructure
Photosensitivity Disorders - complications
Porphyrins - blood
Protoporphyrins - blood
Protoporphyrins - urine
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Description
Summary:A patient with idiopathic sideroblastic anemia and atypical clinical and biochemical findings is described. He had a greatly increased erythrocyte and plasma protoporphyrin, but normal urine and fecal porphyrins. The erythrocyte protoporphyrin had a fluorescence spectrum typical of free protoporphyrin, but caused no photosensitivity. Bone marrow metal chelatase activity was normal. There were no clinical signs of liver disease. The abnormal porphyrin metabolism in this patient is not known though a number of explanations are discussed.
ISSN:0006-4971
1528-0020
DOI:10.1182/blood.V59.3.628.628