The Response of Free Erythrocyte Protoporphyrin to Pyridoxine Therapy in a Patient with Sideroachrestic (Sideroblastic) Anemia

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...

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...

Erythrocyte coproporphyrin and protoporphyrin in ethanol-induced sideroblastic erthropoiesis.

Antibody-mediated acquired sideroblastic anemia: response to cytotoxic therapy.

A 5 1/2-yr-old child developed severe anemia with erythroid hypoplasia and 50% ringed sideroblasts in his bone marrow. A serum inhibitor of erythropoiesis was demonstrated, utilizing syngeneic and autologous bone marrow in a plasma-clot culture system. The IgG fraction of the patient's serum effected similar suppression of erythroid colony formation. Prednisone therapy was ineffective, but foll...

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 ...