Reader TTS

ABSTRACT Erythroid colony (EC) formation and heme biosynthesis were studied in marrow cultures of a 2 year old girl with severe CSA and neutropenia. Bone marrow showed micronormoblastic

erythroid hyperplasia, ringed sideroblasts and decreased granulopoeisis. The patient had not responded to hematinics. and prednisone. EC formation was determined in plasma clot (pc) and

methyl cellulose (mc) culture systems after 7 days incubation by benzidine staining and expressed as CFUE/8×105 marrow cells. In pc cultures, EC(140) were fewer than normal adult controls

(1100).However, cultures in me resulted in exuberant EC growth (1400). Good EC formation (900) also occurred without added erythropoeitin (ep), in contrast to no growth in controls. Addition

of hemin stimulated EC formation (2000) and reversed pyridoxal PO4 inhibition. δ-aminolevulinic acid (ALA) synthetase activity of marrow itself was substantially decreased and heme

oxygenase was increased. However, in me culture ALA synthetase, ALA dehydratase, C- 14ALA incorporation into heme and H-3 leucine incorporation into protein were normal and increased by

addition of hemin. This patient's excellent capacity to synthesize hemoglobin and form EC in me culture, even in the absence of added ep, suggests that CSA may result from an

unfavorable marrow microenvironment. ARTICLE PDF AUTHOR INFORMATION AUTHORS AND AFFILIATIONS * Cornell Univ. Med. Col., North Shore Univ. Hosp., Dept. Ped, Manhasset, NY Joseph Kochen, Nader

Ibrahim, John Lutton & Richard Levere * New York Med. Col. Dept. Med., Valhalla, NY Joseph Kochen, Nader Ibrahim, John Lutton & Richard Levere Authors * Joseph Kochen View author

publications You can also search for this author inPubMed Google Scholar * Nader Ibrahim View author publications You can also search for this author inPubMed Google Scholar * John Lutton

View author publications You can also search for this author inPubMed Google Scholar * Richard Levere View author publications You can also search for this author inPubMed Google Scholar

RIGHTS AND PERMISSIONS Reprints and permissions ABOUT THIS ARTICLE CITE THIS ARTICLE Kochen, J., Ibrahim, N., Lutton, J. _et al._ 827 CONGENITAL SIDEROBLASTIC ANEMIA (CSA): A

MICRO-ENVIRONMENT DEFECT. _Pediatr Res_ 15 (Suppl 4), 580 (1981). https://doi.org/10.1203/00006450-198104001-00852 Download citation * Issue Date: 01 April 1981 * DOI:

https://doi.org/10.1203/00006450-198104001-00852 SHARE THIS ARTICLE Anyone you share the following link with will be able to read this content: Get shareable link Sorry, a shareable link is

not currently available for this article. Copy to clipboard Provided by the Springer Nature SharedIt content-sharing initiative