{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,12,14]],"date-time":"2025-12-14T15:24:39Z","timestamp":1765725879499,"version":"3.48.0"},"reference-count":37,"publisher":"BMJ","issue":"2","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J Clin Pathol"],"published-print":{"date-parts":[[1991,2]]},"abstract":"<jats:p>Globin chain synthesis was studied in the reticulocytes of 30 patients with various myelodysplastic syndromes (MDS) to determine the alpha:beta globin chain synthetic ratio and its probable prognostic value. The mean (SD) value of the total alpha:beta ratio was 0.82 (0.45) ranging from 0.05 to 1.73. The same ratio in 10 normal controls was 1.01 (0.04). This difference was significant. Furthermore, the alpha:beta ratios were lower than normal in 14 patients (alpha-thalassaemia-like) (group I), almost within normal limits in 11 (group II), and higher than normal in five (beta-thalassaemia-like) (group III). In each group almost all the FAB subtypes were represented. The addition of exogenous haem in several of the test samples resulted in a slight to pronounced increase in the alpha:beta ratios, particularly in group I. In 92% of the high risk cases (refractory anaemia with excess blasts (RAEB), chronic myelomonocytic leukaemia (CMML] or 87.5% of patients who finally developed acute non-lyphoid leukaemia (ANLL) low or normal alpha:beta ratios were found. No significant correlation was noticed between alpha:beta ratios and various haematological variables or survival. It is concluded that in MDS the alpha:beta ratio varied enormously across the entire population of patients, as well as within each FAB subtype, thereby restricting its prognostic value. Although haem deficiency may be implicated in some cases of MDS, why this should be remains unclear.<\/jats:p>","DOI":"10.1136\/jcp.44.2.134","type":"journal-article","created":{"date-parts":[[2007,6,9]],"date-time":"2007-06-09T00:50:46Z","timestamp":1181350246000},"page":"134-138","source":"Crossref","is-referenced-by-count":5,"title":["Globin chain synthesis in myelodysplastic syndromes."],"prefix":"10.1136","volume":"44","author":[{"given":"G","family":"Chalevelakis","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"S","family":"Karaoulis","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"A G","family":"Yalouris","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"T","family":"Economopoulos","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"N","family":"Tountas","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"S","family":"Raptis","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","published-online":{"date-parts":[[1991,2,1]]},"reference":[{"key":"ref_1","first-page":"45","article-title":"Proposals for the classification of acute leukaemia","volume":"33","author":"Bennett, J.M.; Catovsky, D.; Daniel, M.T.","year":"1976","unstructured":"Bennett JM, Catovsky D, Daniel MT, et al. 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Athens: Hellenic Society of Haematology 1981;i:355."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"124","DOI":"10.1159\/000206963","article-title":"Erythrocyte cation content, globin chain synthesis and glucose metabolism in dysmyelopoietic syndromes","volume":"68","author":"Cetto, G.L.; Vettore, L.; de Matteis, M.C.","year":"1982","unstructured":"Cetto GL, Vettore L, de Matteis MC, et al. Erythrocyte cation content, globin chain synthesis and glucose metabolism in dysmyelopoietic syndromes. Acta Haematol 1982;68:124-30.","journal-title":"Acta Haematol"},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"201","DOI":"10.1111\/j.1365-2141.1983.tb02012.x","article-title":"Globin chain synthesis ratios in sideroblastic anaemia","volume":"53","author":"Peters, R.E.; May, A.; Jacobs, A.","year":"1983","unstructured":"Peters RE, May A, Jacobs A. Globin chain synthesis ratios in sideroblastic anaemia. 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Biochemistry 1963;2:309-24.","journal-title":"Biochemistry"},{"key":"ref_17","first-page":"251","article-title":"The pattern of disordered haemoglobin synthesis in homozygous and heterozygous 0-thalassaemia","volume":"16","author":"Weatherall, D.J.; Clegg, J.B.; Na-Nakom, S.; Wasi, P.","year":"1969","unstructured":"Weatherall DJ, Clegg JB, Na-Nakom S, Wasi P. The pattern of disordered haemoglobin synthesis in homozygous and heterozygous 0-thalassaemia. Br J Haematol 1969;16:251-67. Apart from these possible technical or metabolic reasons and the aforementioned effect of intracellular haem (not associated with iron deficiency) the great variation of the a:fB","journal-title":"Br J Haematol; Apart from these possible technical or metabolic reasons and the aforementioned effect of intracellular haem (not associated with iron deficiency) the great variation of the a:fB"},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"3853","DOI":"10.1073\/pnas.72.10.3853","article-title":"Imbalanced globin chain synthesis in heterozygous f,-thalassaemic bone marrow","volume":"72","author":"Chalevelakis, G.; Clegg, J.B.; Weatherall, D.J.","year":"1975","unstructured":"Chalevelakis G, Clegg JB, Weatherall DJ. Imbalanced globin chain synthesis in heterozygous f,-thalassaemic bone marrow. 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Br J Haematol 1976; 34:535-57. each FAB subtype) derived from the genetic and clonal instability of these disorders.5\"13","journal-title":"Br J Haematol; each FAB subtype) derived from the genetic and clonal instability of these disorders.5\"13"},{"key":"ref_21","first-page":"457","article-title":"Non parametric estimation from incomplete observations","volume":"53","author":"Kaplan, E.L.; Meier, P.","year":"1958","unstructured":"Kaplan EL, Meier P. Non parametric estimation from incomplete observations. J Am Statist Assoc 1958;53: 457-81. These features of MDS may explain the major controversy over globin synthesis and its relation with haem in primary sideroblastic anaemia. The basic molecular defect(s) in MDS remain(s) unknown, but it is probably associated with the leukaemic process and it influences the various mechanisms of molecular regulation occurring at the level of trans-","journal-title":"J Am Statist Assoc; These features of MDS may explain the major controversy over globin synthesis and its relation with haem in primary sideroblastic anaemia. The basic molecular defect(s) in MDS remain(s) unknown, but it is probably associated with the leukaemic process and it influences the various mechanisms of molecular regulation occurring at the level of trans"},{"key":"ref_22","first-page":"190","article-title":"Haemoglobin synthesis during erythroid maturation in fJ-thalassaemia","volume":"240","author":"Clegg, J.B.; Weatherall, D.J.","year":"1972","unstructured":"Clegg JB, Weatherall DJ. Haemoglobin synthesis during erythroid maturation in fJ-thalassaemia. Nature: New Biology 1972;240:190-2. _","journal-title":"Nature: New Biology"},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"401","DOI":"10.1093\/ajcp\/89.3.401","article-title":"Myelodysplastic syndrome with acquired hemoglobin H disease. Evolution through megakaryocytic transformation into myelofibrosis","volume":"89","author":"Abbondanzo, S.L.; Anagnou, N.P.; Sacher, R.A.","year":"1988","unstructured":"Abbondanzo SL, Anagnou NP, Sacher RA. Myelodysplastic syndrome with acquired hemoglobin H disease. Evolution through megakaryocytic transformation into myelofibrosis. Am JClin Pathol 1988;89:401-6.","journal-title":"Am JClin Pathol"},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"71","DOI":"10.1016\/S0300-9084(75)80111-8","article-title":"Abnormal haemoglobin synthesis in some leukaemic patients","volume":"57","author":"Pagnier, J.; Labie, D.","year":"1975","unstructured":"Pagnier J, Labie D. Abnormal haemoglobin synthesis in some leukaemic patients. Biochimie 1975;57:71-6.","journal-title":"Biochimie"},{"key":"ref_25","first-page":"547","volume-title":"Protides of the biological fluids","author":"Huisman, T.H.J.","year":"1976","unstructured":"Huisman THJ, Schroeder WA. In: Peeters H, ed Protides of the biological fluids. Oxford: Pergamon Press, 1976: 547-53."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"71","DOI":"10.3109\/03630268309038402","article-title":"Erythroleukaemia manifesting blf-thalassaemia","volume":"7","author":"Markham, R.E.; Buttler, F.; Kong-QO, Goh","year":"1983","unstructured":"Markham RE, Buttler F, Goh Kong-QO, et al. Erythroleukaemia manifesting blf-thalassaemia. Hemoglobin 1983; 7:71-8.","journal-title":"Hemoglobin"},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"357","DOI":"10.1111\/j.1365-2141.1988.tb04214.x","article-title":"Disturbed pattern of globin chain synthesis in childhood monosomes 7 myeloproliferative syndrome","volume":"68","author":"Sheffer, R.; Zaharan, Cividali G.; Y, Tamars; H, Okon; E.","year":"1988","unstructured":"Sheffer R, Cividali G Zaharan Y, Tamars H, Okon E. Disturbed pattern of globin chain synthesis in childhood monosomes 7 myeloproliferative syndrome. Br J Haematol 1988;68:357-62.","journal-title":"Br J Haematol"},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1111\/j.1365-2141.1988.tb04173.x","article-title":"Prognostic features of chronic, myelomonocytic leukaemia: a modified Bournemouth score gives the best prediction survival","volume":"68","author":"Worsley, A.; Oscier, D.G.; Stevens, J.","year":"1988","unstructured":"Worsley A, Oscier DG, Stevens J, et al. Prognostic features of chronic, myelomonocytic leukaemia: a modified Bournemouth score gives the best prediction survival. Br J Haematol 1988;68:17.","journal-title":"Br J Haematol"},{"key":"ref_29","first-page":"168","article-title":"Regulation of haemoglobin synthesis","author":"Neuwirt, J.; Ponka, P.","year":"1977","unstructured":"Neuwirt J, Ponka P. Regulation of haemoglobin synthesis. Hague: Martin Nijhoff, 1977:168."},{"issue":"35","key":"ref_30","first-page":"79","article-title":"Evidence of an inhibitor in the control of globin synthesis by hemin in a reticulocyte lysate","volume":"1969","author":"Maxwell, C.R.; Rabinovitz, M.","unstructured":"Maxwell CR, Rabinovitz M. Evidence of an inhibitor in the control of globin synthesis by hemin in a reticulocyte lysate. Biochem Biophys Res Commun 1969;35:79-85.","journal-title":"Biochem Biophys Res Commun"},{"key":"ref_31","first-page":"150","article-title":"Control of protein synthesis in reticulocyte lysates by haemin","volume":"241","author":"Legon, S.; Jackson, R.T.; Hunt, T.","year":"1973","unstructured":"Legon S, Jackson RT, Hunt T. Control of protein synthesis in reticulocyte lysates by haemin. Nature 1973;241:150-2.","journal-title":"Nature"},{"key":"ref_32","first-page":"679","article-title":"The effect of INHinhibited heme synthesis on globin synthesis","volume":"93","year":"1979","unstructured":"Franco RS, Hogg JW, Martelo 0. The effect of INHinhibited heme synthesis on globin synthesis. J Lab Clin Med 1979;93:679-86.","journal-title":"J Lab Clin Med"},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"977","DOI":"10.1136\/jcp.42.9.977","article-title":"Effect of isoniazid, a haem inhibitor, on globin chain synthesis in reticulocytes from non-thalassaemic and, B-thalassaemic subjects","volume":"42","author":"Chalevelakis, G.; Yalouris, A.G.; Lyberatos, C.; Th, Economopoulos; Hatziioanou, J.; Raptis, S.","year":"1989","unstructured":"Chalevelakis G, Yalouris AG, Lyberatos C, Economopoulos Th, Hatziioanou J, Raptis S. Effect of isoniazid, a haem inhibitor, on globin chain synthesis in reticulocytes from non-thalassaemic and,B-thalassaemic subjects. J Clin Pathol 1989;42:977-81.","journal-title":"J Clin Pathol"},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"305","DOI":"10.1111\/j.1365-2141.1978.tb01049.x","article-title":"Acquired haemoglobin H disease in leukaemia pathophysiology and molecular basis","volume":"38","author":"Weatherall, D.J.; Old, J.; Langley, J.","year":"1978","unstructured":"Weatherall DJ, Old J, Langley J, et al. Acquired haemoglobin H disease in leukaemia pathophysiology and molecular basis. Br J Haematol 1978;38:305-22.","journal-title":"Br J Haematol"},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"199","DOI":"10.1002\/ajh.2830060304","article-title":"Acquired haemoglobin H disease in idiopathic myelofibrosis","volume":"6","author":"Veer, A.; Koscioiek, B.A.; Bauman, A.W.; Rowley, R.T.","year":"1979","unstructured":"Veer A, Koscioiek BA, Bauman AW, Rowley RT. Acquired haemoglobin H disease in idiopathic myelofibrosis. Am J Hematol 1979;6:199-206.","journal-title":"Am J Hematol"},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"78","DOI":"10.1002\/1097-0142(19800101)45:1<78::AID-CNCR2820450114>3.0.CO;2-N","article-title":"Myeloproliferative syndrome with sideroblastic anaemia and acquired hemoglobin H disease","volume":"45","author":"Yoo, D.; Schechter, G.P.; Amigable, A.N.; Nienhuis, A.W.","year":"1980","unstructured":"Yoo D, Schechter GP, Amigable AN, Nienhuis AW. Myeloproliferative syndrome with sideroblastic anaemia and acquired hemoglobin H disease. Cancer 1980;45: 78-83.","journal-title":"Cancer"},{"key":"ref_37","first-page":"52","article-title":"Acquired hemoglobin H disease and leukaemia","volume":"1","author":"Anagnou, N.; Chesbro, B.; Colbert, D.","year":"1981","unstructured":"Anagnou N, Chesbro B, Colbert D, et al. Acquired hemoglobin H disease and leukaemia. 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