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تعداد آیتم قابل مشاهده باقیمانده : 2 مورد

Causes of abnormal hemoglobin A2 levels

Causes of abnormal hemoglobin A2 levels
Reduced Hb A2 Increased Hb A2
Condition Comments Condition Comments
Alpha thalassemia Reduction correlates with severity of thalassemia Sickle cell disease Artifact of some HPLC systems
Alpha globin gene variants Hybrid Hb A2 might not separate Beta thalassemia Typically 4 to 6%; rarely up to 10% with 5' HBB gene deletions
Delta globin gene variants Variant can be poorly expressed Sickle cell-beta thalassemia >3.5%; may be artifact of some HPLC systems
Delta-beta thalassemia Half normal in heterozygotes Sickle cell disease with alpha thalassemia Typically 4 to 5%; due to a post-translational effect
Delta thalassemia Usually approximately half normal Sickle cell trait Artifact of some HPLC systems
Gene deletional HPFH Half normal in heterozygotes Unstable hemoglobins  
Hb Lepore Half normal in heterozygotes Megaloblastic anemia Modest increases
Sideroblastic anemia Due to impaired alpha globin synthesis Hereditary spherocytosis Not uniformly elevated
Iron deficiency Reduction correlates with severity of iron deficiency KLF1 gene mutations Lower than in beta thalassemia
Acute myeloid leukemia Minor reduction Congenital dyserythropoietic anemia, type 1 Lower than in beta thalassemia
Myelodysplastic syndrome Only reduced in a minority of cases Hyperthyroidism Lower than in beta thalassemia
Juvenile chronic granulocytic leukemia Only reduced in a minority of cases with increased Hb F Zidovudine treatment for HIV infection Lower than in beta thalassemia
Hb A2 is a normal adult hemoglobin consisting of two alpha chains and two delta chains (alpha2/delta2). Normal Hb A2 levels are approximately 2.8% (range, 2.1 to 3.1%). Increased or reduced Hb A2 levels may indicated one of the conditions listed in the table (inherited conditions are listed first, followed by acquired conditions). Refer to UpToDate for further discussions of these disorders and their evaluation.
HPLC: high-performance liquid chromatography; HPFH: hereditary persistence of fetal hemoglobin; Hb F: fetal hemoglobin.
Adapted with permission from:
  1. Steinberg MH, Nagel RL. Hemoglobins of the embryo, fetus, and adult. In: Disorders of Hemoglobin: Genetics, Pathophysiology, and Clinical Management, 2nd ed, Steinberg MH, Forget BG, Higgs DR, Weatherall DJ (Eds), Cambridge University Press, New York 2009. Copyright © 2009 Cambridge University Press.
  2. Steinberg MH, Rodgers GP. HbA2: Biology, clinical relevance and a possible target for ameliorating sickle cell disease. Br J Haematol 2015; 170(6):781-7. https://onlinelibrary.wiley.com/doi/abs/10.1111/bjh.13570. Copyright © 2015 John Wiley & Sons Inc.
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