PMID- 6690472
OWN - NLM
STAT- MEDLINE
DCOM- 19840220
LR  - 20181113
IS  - 0021-9738 (Print)
IS  - 0021-9738 (Linking)
VI  - 73
IP  - 1
DP  - 1984 Jan
TI  - Concurrent sickle cell anemia and alpha-thalassemia. Effect on pathological 
      properties of sickle erythrocytes.
PG  - 116-23
AB  - The concurrence of sickle cell anemia and alpha-thalassemia results in less 
      severe hemolytic anemia apparently as a result of reduced intraerythrocytic 
      concentration of hemoglobin S and its retarded polymerization. We have evaluated 
      the effect of alpha-globin gene number on several interrelated properties of 
      sickle erythrocytes (RBC) that are expected to correlate with the hemolytic and 
      rheologic consequences of sickle cell disease. The irreversibly sickled cell 
      number, proportion of very dense sickle RBC, and diminished deformability of 
      sickle RBC each varied directly with alpha-globin gene number. Sickle RBC density 
      was a direct function of the mean corpuscular hemoglobin concentration (MCHC). 
      Even in nonsickle RBC, alpha-globin gene number varied directly with RBC density. 
      Despite differences in alpha-globin gene number, sickle RBC of the same density 
      had the same degree of deformability and dehydration. These data indicate that 
      the fundamental effect of alpha-thalassemia is to inhibit the generation of 
      sickle RBC having high density and MCHC, and that the other beneficial effects of 
      sickle RBC are secondary to this process. The less consistent effect on overall 
      clinical severity reported for subjects with this concurrence may reflect an 
      undefined detrimental effect of alpha-thalassemia, possibly on the whole blood 
      viscosity or on sickle RBC membrane-mediated adherence phenomena.
FAU - Embury, S H
AU  - Embury SH
FAU - Clark, M R
AU  - Clark MR
FAU - Monroy, G
AU  - Monroy G
FAU - Mohandas, N
AU  - Mohandas N
LA  - eng
GR  - AM 26263/AM/NIADDK NIH HHS/United States
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  - J Clin Invest
JT  - The Journal of clinical investigation
JID - 7802877
RN  - 0 (Hemoglobin, Sickle)
RN  - 0 (Macromolecular Substances)
RN  - 9004-22-2 (Globins)
SB  - IM
MH  - Anemia, Sickle Cell/*blood/*complications/genetics
MH  - Erythrocyte Count
MH  - Erythrocyte Indices
MH  - Erythrocytes, Abnormal/*pathology/physiology
MH  - Genes, Regulator
MH  - Globins/genetics
MH  - Hemoglobin, Sickle/metabolism
MH  - Humans
MH  - Macromolecular Substances
MH  - Osmotic Fragility
MH  - Thalassemia/*blood/complications/genetics
PMC - PMC424978
EDAT- 1984/01/01 00:00
MHDA- 1984/01/01 00:01
PMCR- 1984/01/01
CRDT- 1984/01/01 00:00
PHST- 1984/01/01 00:00 [pubmed]
PHST- 1984/01/01 00:01 [medline]
PHST- 1984/01/01 00:00 [entrez]
PHST- 1984/01/01 00:00 [pmc-release]
AID - 10.1172/JCI111181 [doi]
PST - ppublish
SO  - J Clin Invest. 1984 Jan;73(1):116-23. doi: 10.1172/JCI111181.