Sideroblastic anemia with markedly increased free erythrocyte protoporphyrin without dermal photosensitivity

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 protoporphyrin, but caused no photosensitivity. Bone marrow metal chelatase activity was normal. There were n...

The response of free erythrocyte protoporphyrin to pyridoxine therapy in a patient with sideroachrestic (sideroblastic) anemia.

By G. R. LEE, G. E. CARTWRIGHT AND M. M. \VINTROBE F’ EILMEYER has applied the term “sideroachrestic anemia” to conditions characterized by the association of hypochromic anemia with excessive iron storage.’ In referring to the same syndrome, Mollin and others2 prefer the designation “sideroblastic anemia,” a term which emphasizes the importance of abnormal iron accumulation in erythrohlasts. C...

Free erythrocyte protoporphyrin.

Porphyrins are widely distributed in living cells throughout nature and play essential roles in various metabolic processes such as photosynthesis, transportation of oxygen and cellular respiration. In man the most important of the porphyrins is protoporphyrin 9, type III, which in combination with iron and specific proteins forms such compounds as hemoglobin, myoglobin, cytochrome, peroxidase ...

Erythrocyte coproporphyrin and protoporphyrin in ethanol-induced sideroblastic erthropoiesis.

Sideroblastic anemia.

When first defined 50 years ago, sideroblastic anemia (SA) was already recognized to occur in heterogeneous settings, including as familial or acquired disease. The spectrum of SA has since become considerably expanded with respect to distinct clinical phenotypes as well as discrete causes. The singular feature that typifies all forms of SA and is required for initial diagnosis is the presence ...