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Related Collections Red Cells Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  Blood, 1 October 2000, Vol. 96, No. 7, pp. 2606-2612 RED CELLS A correlation of erythrokinetics, ineffective erythropoiesis, and erythroid precursor apoptosis in Thai patients with thalassemia Pensri Pootrakul, Pornpan Sirankapracha, Surai Hemsorach, Wanna Moungsub, Rawiprapa Kumbunlue, Anong Piangitjagum, Prawase Wasi, Lisa Ma, and Stanley L. Schrier From the Thalassemia Research Center, Institute of Sciences and Technology for Research and Development, Mahidol University, Salaya Campus, Nakornprathom; the Faculty of Medicine, Siriraj Hospital, Mahidol University; the Faculty of Tropical Medicine, Mahidol University, Bangkok, Thailand; and the Division of Hematology, Department of Medicine, Stanford University School of Medicine, Stanford, CA. The variety of patients with thalassemia in Thailand offers an opportunity to fully characterize the kinetic causes of the anemia and to study apoptosis of marrow erythroid precursors as a possible factor contributing to its severity. Kinetic studies showed that in hemoglobin H (HbH) disease, the extent of hemolysis, as well as the minimally ineffective erythropoiesis, usually falls within the compensatory capacity of normal erythropoiesis; therefore, anemia in patients with HbH partly represents a failure to expand erythropoiesis adequately. Hemoglobin Constant Spring (HbCS), a common variant of  thalassemia in Bangkok, causes more severe hemolysis and a distinct increase in ineffective erythropoiesis. Ineffective erythropoiesis plays a much more prominent role in  thalassemia/hemoglobin E (-thal/HbE) disease, in which the variability of the anemia is puzzling. We compared mild and severe cases and found that patients with severe disease had a maximal marrow erythropoietic response that failed to compensate for very short survival of red blood cells and a marked quantitative increase in ineffective erythropoiesis. Analysis of apoptosis of marrow erythroid precursors done both on shipped samples and in Bangkok showed a moderate increase in HbH disease, consistent with the small increase in ineffective erythropoiesis. In patients with homozygous HbCS, there was a further increase in apoptosis, consistent with the additional increase in ineffective erythropoiesis. Patients with -thal/HbE disease had the most ineffective erythropoiesis and the most erythroid apoptosis. Thus, it appears that -chain deposition in erythroid precursors, either A or cs, leads to accelerated apoptosis and ineffective erythropoiesis. © 2000 by The American Society of Hematology.   CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? 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