PMID- 3965046
OWN - NLM
STAT- MEDLINE
DCOM- 19850128
LR  - 20220330
IS  - 0006-4971 (Print)
IS  - 0006-4971 (Linking)
VI  - 65
IP  - 1
DP  - 1985 Jan
TI  - Hemoglobin S polymerization: primary determinant of the hemolytic and clinical 
      severity of the sickling syndromes.
PG  - 183-9
AB  - We examined the extent to which the intracellular polymerization of sickle 
      hemoglobin (HbS) can account for the severity of anemia and of vaso-occlusive 
      manifestations in the various sickling syndromes. Polymer formation in sickle 
      cell disease depends principally on the intraerythrocytic hemoglobin composition 
      and concentration. In our studies, the polymer fraction in sickle red cells was 
      determined from reported mean values for hemoglobin composition and mean 
      corpuscular hemoglobin concentration (MCHC) in 12 groups of patients with sickle 
      hemoglobinopathies (homozygotes for HbS, with and without coexistent 
      alpha-thalassemia or various forms of the hereditary persistence of fetal 
      hemoglobin [HPFH], beta+-, beta 0-, and delta beta-thalassemia, and heterozygotes 
      for HbS with HbA). The calculated HbS polymer fractions at full deoxygenation and 
      at physiologic oxygen saturation values were closely correlated with mean blood 
      hemoglobin concentrations. In addition, polymer fraction correlated with the 
      ranking of the sickling syndromes by vaso-occlusive severity. We find that 
      polymer fraction accounts for about 80% of the variability in hemolytic and 
      clinical severity. The method of analysis presented here provides a quantitative 
      and systematic means of assessing the role of polymer formation in the 
      pathophysiologic manifestations of the sickling syndromes. Our results support 
      the hypothesis that the intracellular polymerization of HbS is the primary 
      determinant of the severity of both anemia and clinical symptomatology in the 
      sickle hemoglobinopathies.
FAU - Brittenham, G M
AU  - Brittenham GM
FAU - Schechter, A N
AU  - Schechter AN
FAU - Noguchi, C T
AU  - Noguchi CT
LA  - eng
PT  - Journal Article
PL  - United States
TA  - Blood
JT  - Blood
JID - 7603509
RN  - 0 (Hemoglobin, Sickle)
RN  - 0 (Macromolecular Substances)
RN  - S88TT14065 (Oxygen)
SB  - IM
MH  - Anemia, Sickle Cell/*blood/genetics/physiopathology
MH  - Erythrocytes, Abnormal/metabolism
MH  - Erythropoiesis
MH  - Genetic Carrier Screening
MH  - Hemoglobin, Sickle/analysis/*metabolism
MH  - *Hemolysis
MH  - Humans
MH  - Macromolecular Substances
MH  - Oxygen/blood
MH  - Sickle Cell Trait/blood
MH  - Syndrome
EDAT- 1985/01/01 00:00
MHDA- 1985/01/01 00:01
CRDT- 1985/01/01 00:00
PHST- 1985/01/01 00:00 [pubmed]
PHST- 1985/01/01 00:01 [medline]
PHST- 1985/01/01 00:00 [entrez]
AID - S0006-4971(20)83740-9 [pii]
PST - ppublish
SO  - Blood. 1985 Jan;65(1):183-9.