Concurrent Sickle-Cell Anemia and α-Thalassemia

Authors:
  • SEStephen H. Embury
    Stephen H. Embury
  • ADAndree M. Dozy
    Andree M. Dozy
  • JMJudy Miller
    Judy Miller
  • JDJ. R. Davis
    J. R. Davis
  • KKKlara M. Kleman
    Klara M. Kleman
  • HPHaiganoush Preisler
    Haiganoush Preisler
  • EVElliott Vichinsky
    Elliott Vichinsky
  • WLWilliam N. Lande
    William N. Lande
  • BLBertram H. Lubin
    Bertram H. Lubin
  • YKY. W. Kan
    Y. W. Kan
  • WMWilliam C. Mentzer
    William C. Mentzer
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Abstract
We studied 47 patients with sickle-cell anemia to determine the effect of α-thalassemia on the severity of their hemolytic anemia. We diagnosed α-thalassemia objectively by using α-globin-gene mapping to detect α-globin-gene deletions, studying 25 subjects with the normal four α-globin genes, 18 with three, and four with two. The mean hemoglobin, hematocrit, and absolute reticulocyte levels (±S.D.) were 7.9±0.9 g per deciliter (4.9±0.6 mmol per liter), 22.9±2.9 per cent, and 501,000±126,000 per cubic millimeter, respectively, in the non-thalassemic group; 9.8±1.6 g per deciliter (6.1±1.0 mmol per liter), 29.0±5.0 per cent, and 361,000±51,000 per cubic millimeter in the group with three α-globin genes; and 9.2±1.0 g per deciliter (5.7±0.6 mmol per liter), 27.5±3.0 per cent, and 100,000±15,000 per cubic millimeter in the group with two α-globin genes. Deletion of α-globin genes was also accompanied by a decreased mean corpuscular hemoglobin concentration (MCHC) in post-reticulocyte erythrocytes and by increased hemoglobin F levels. The decreased intraerythrocytic hemoglobin S concentration and elevated hemoglobin F levels associated with α-thalassemia appear to diminish the degree of hemolytic anemia found in sickle-cell disease. (N Engl J Med. 1982; 306:270–4.)

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