A correlation of erythrokinetics, ineffective erythropoiesis, and erythroid precursor apoptosis in thai patients with thalassemia.

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  • Additional Information
    • Source:
      Publisher: Elsevier Country of Publication: United States NLM ID: 7603509 Publication Model: Print Cited Medium: Print ISSN: 0006-4971 (Print) Linking ISSN: 00064971 NLM ISO Abbreviation: Blood Subsets: MEDLINE
    • Publication Information:
      Publication: 2021- : [New York] : Elsevier
      Original Publication: New York, Grune & Stratton [etc.]
    • Subject Terms:
      Apoptosis* ; Erythrocyte Aging* ; Erythropoiesis*; Erythroid Precursor Cells/*pathology ; Thalassemia/*blood; Adolescent ; Adult ; Bone Marrow/pathology ; Hemoglobin E/analysis ; Hemoglobins, Abnormal/analysis ; Hemolysis ; Humans ; Kinetics ; Middle Aged ; Thailand ; alpha-Thalassemia/blood ; beta-Thalassemia/blood
    • Abstract:
      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 alpha thalassemia in Bangkok, causes more severe hemolysis and a distinct increase in ineffective erythropoiesis. Ineffective erythropoiesis plays a much more prominent role in beta thalassemia/hemoglobin E (beta-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 beta-thal/HbE disease had the most ineffective erythropoiesis and the most erythroid apoptosis. Thus, it appears that alpha-chain deposition in erythroid precursors, either alpha(A) or alpha(cs), leads to accelerated apoptosis and ineffective erythropoiesis.
    • Grant Information:
      HL RO1-34408 United States HL NHLBI NIH HHS; R01-DK13682 United States DK NIDDK NIH HHS
    • Accession Number:
      0 (Hemoglobins, Abnormal)
      9034-61-1 (Hemoglobin E)
      9066-22-2 (Hemoglobin Constant Spring)
    • Publication Date:
      Date Created: 20000926 Date Completed: 20001025 Latest Revision: 20210218
    • Publication Date:
      20250114
    • Accession Number:
      11001918