The response of free erythrocyte protoporphyrin to pyridoxine therapy in a patient with sideroachrestic (sideroblastic) anemia.
نویسندگان
چکیده
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. Classifications of the reported cases have been proposed.1’2 The patients in whom “sideroachrcstic anemia” has been detected differ from one another in several important respects, including the response to pyridoxine therapy,3’4 the association with underlying disease,5 and the cvidence of familial transmission of the disorder.#{176} It has been suggested”7 that these varied disorders have in common the presence of a metabolic lesion in the hemoglobin synthetic pathways. We have studied a patient with acquired sideroachrcstic ( sideroblastic) anemia and carcinoma of the prostate in whom a remarkable amount of protoporphyrin accumulated within his erythrocytes during pyridoxine therapy. Since this observation contributed to the understanding of the biochemical defects present in this patient, the case report is presented. A preliminary report was published elsewhere.8
منابع مشابه
The Response of Free Erythrocyte
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...
متن کاملBone marrow delta-aminolaevulinate synthase deficiency in a female with congenital sideroblastic anemia.
Heme biosynthesis was examined in erythroid tissue of a 4-yr-old girl with severe sideroblastic anemia since infancy, as documented by the presence of intramitochondrial deposits of iron in erythroblasts. Free red cell protoporphyrin, urinary porphyrins, and activities of erythrocyte porphobilinogen synthase, uroporphyrinogen 1 synthase, aspartate aminotransferase, and pyridoxine kinase were no...
متن کاملSideroblastic 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...
متن کاملAbnormal Iron Incorporation, Survival, Protoporphyrin Content and Fluorescence of One Red Cell Population in Preleukemic Sideroblastic Anemia
The dimorphic blood picture in sideroblastic anemia suggests the coexistence of at least two types of erythrocyte, the populations with the smaller RBCs being responsible for the crucial abnormality, namely the impairment of iron incorporation into protoporphyrin. In a patient with sideroblastic anemia who subsequently developed acute myelocytic leukemia, we found a light and a heavy cell popul...
متن کاملAbnormal iron incorporatin, survival protoporphyrin content and fluorescence of one red cell population in preleukemic sideroblastic anemia.
The dimorphic blood picture in sideroblastic anemia suggests the coexistence of at least two types of erythrocyte, the populations with the smaller RBCs being responsible for the crucial abnormality, namely the impairment of iron incorporation into protoporphyrin. In a patient with sideroblastic anemia who subsequently developed acute myelocytic leukemia, we found a light and a heavy cell popul...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید
ثبت ناماگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید
ورودعنوان ژورنال:
- Blood
دوره 27 4 شماره
صفحات -
تاریخ انتشار 1966