PMID- 6172710
OWN - NLM
STAT- MEDLINE
DCOM- 19820313
LR  - 20220409
IS  - 0028-4793 (Print)
IS  - 0028-4793 (Linking)
VI  - 306
IP  - 5
DP  - 1982 Feb 4
TI  - Concurrent sickle-cell anemia and alpha-thalassemia: effect on severity of 
      anemia.
PG  - 270-4
AB  - We studied 47 patients with sickle-cell anemia to determine the effect of 
      alpha-thalassemia on the severity of their hemolytic anemia. We diagnosed 
      alpha-thalassemia objectively by using alpha-globin-gene mapping to detect 
      alpha-globin-gene deletions, studying 25 subjects with the normal four 
      alpha-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 alpha-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 
      alpha-globin genes. Deletion of alpha-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 alpha-thalassemia appear to diminish the degree of hemolytic 
      anemia found in sickle-cell disease.
FAU - Embury, S H
AU  - Embury SH
FAU - Dozy, A M
AU  - Dozy AM
FAU - Miller, J
AU  - Miller J
FAU - Davis, J R Jr
AU  - Davis JR Jr
FAU - Kleman, K M
AU  - Kleman KM
FAU - Preisler, H
AU  - Preisler H
FAU - Vichinsky, E
AU  - Vichinsky E
FAU - Lande, W N
AU  - Lande WN
FAU - Lubin, B H
AU  - Lubin BH
FAU - Kan, Y W
AU  - Kan YW
FAU - Mentzer, W C
AU  - Mentzer WC
LA  - eng
GR  - HL 20985/HL/NHLBI NIH HHS/United States
PT  - Journal Article
PT  - Research Support, U.S. Gov't, P.H.S.
PL  - United States
TA  - N Engl J Med
JT  - The New England journal of medicine
JID - 0255562
RN  - 0 (Hemoglobins)
RN  - 9004-22-2 (Globins)
RN  - 9034-53-1 (Hemoglobin A2)
RN  - 9034-63-3 (Fetal Hemoglobin)
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Age Factors
MH  - Anemia, Sickle Cell/blood/*complications/genetics
MH  - Child
MH  - Child, Preschool
MH  - Erythrocyte Count
MH  - Fetal Hemoglobin/analysis
MH  - Genes
MH  - Globins/genetics
MH  - Hematocrit
MH  - Hemoglobin A2/analysis
MH  - Hemoglobins/analysis
MH  - *Hemolysis
MH  - Humans
MH  - Reticulocytes
MH  - Thalassemia/blood/*complications/genetics
EDAT- 1982/02/04 00:00
MHDA- 2001/03/28 10:01
CRDT- 1982/02/04 00:00
PHST- 1982/02/04 00:00 [pubmed]
PHST- 2001/03/28 10:01 [medline]
PHST- 1982/02/04 00:00 [entrez]
AID - 10.1056/NEJM198202043060504 [doi]
PST - ppublish
SO  - N Engl J Med. 1982 Feb 4;306(5):270-4. doi: 10.1056/NEJM198202043060504.