PMID- 2441597
OWN - NLM
STAT- MEDLINE
DCOM- 19870918
LR  - 20190820
IS  - 0361-8609 (Print)
IS  - 0361-8609 (Linking)
VI  - 25
IP  - 4
DP  - 1987 Aug
TI  - Effects of alpha-thalassemia-2 on the developmental changes of hematological 
      values in children with sickle cell disease from Georgia.
PG  - 389-400
AB  - The hematology and pathophysiology of sickle cell disease during the postnatal 
      development of younger hemoglobin (Hb) S homozygotes (SS) could be considerably 
      affected by a variability of alpha globin gene numbers. We have documented 
      longitudinal developmental changes of hematological values and hemoglobin 
      composition on 147 patients with SS (alpha alpha/alpha alpha), 64 with SS 
      (-alpha/alpha alpha), and 9 with SS (-alpha/-alpha) between the ages of 1 and 15 
      years. Non-steady-state data were excluded from these analyses. The number and 
      organization of alpha globin genes was established by gene mapping. As 
      anticipated, mean corpuscular volume and erythrocyte counts correlated with alpha 
      globin gene numbers throughout the 15-year age interval. On the other hand, SS 
      children with alpha alpha/alpha alpha, -alpha/alpha alpha, -alpha/-alpha had 
      similar hemoglobin concentrations up to the ages of 5-10 years. Around the age of 
      7, the SS patients with -alpha/-alpha developed a higher Hb concentration than 
      that of the SS (-alpha/alpha alpha), which in turn was higher than that of the SS 
      (alpha alpha/alpha alpha). The emergence of this difference coincided with a 
      developmental increase of the mean corpuscular hemoglobin concentration (MCHC) in 
      patients with SS (alpha alpha/alpha alpha) and the decline of Hb F % under 15%. 
      This newly observed developmental change of the MCHC could lead to increased 
      hemolysis and anemia after the age of 5-10 years. It occurs to a smaller extent 
      among SS (-alpha/alpha alpha) or not at all among SS (-alpha/-alpha) such that 
      these two categories of patients have less severe hemolysis and higher hemoglobin 
      levels at older ages. Although the proportion of Hb F was independent of alpha 
      globin gene numbers, the absence of Hb Bart's suggested that alpha-thalassemia 
      promotes the intracellular assembly of Hb F over Hb S tetramers. Thus, the 
      interaction of alpha-thalassemia and Hb F in young SS patients may be more 
      complex than revealed by Hb F levels in cell lysates. Among older SS children 
      (greater than 7 years) alpha-thalassemia and Hb F levels exceeding 15% appear to 
      have additive effects in diminishing the rate of hemolysis.
FAU - Felice, A E
AU  - Felice AE
FAU - McKie, K M
AU  - McKie KM
FAU - Cleek, M P
AU  - Cleek MP
FAU - Marino, E M
AU  - Marino EM
FAU - Kutlar, A
AU  - Kutlar A
FAU - McKie, V C
AU  - McKie VC
LA  - eng
GR  - HLB 15158/HL/NHLBI NIH HHS/United States
PT  - Journal Article
PT  - Research Support, U.S. Gov't, Non-P.H.S.
PT  - Research Support, U.S. Gov't, P.H.S.
PL  - United States
TA  - Am J Hematol
JT  - American journal of hematology
JID - 7610369
RN  - 0 (Hemoglobin, Sickle)
RN  - 9034-63-3 (Fetal Hemoglobin)
SB  - IM
MH  - Adolescent
MH  - Aging/blood
MH  - Anemia, Sickle Cell/*blood/genetics/physiopathology
MH  - Child
MH  - Child, Preschool
MH  - Chromosome Mapping
MH  - Erythrocyte Count
MH  - *Erythrocyte Indices
MH  - Fetal Hemoglobin/analysis
MH  - Georgia
MH  - Hematocrit
MH  - Hemoglobin, Sickle/analysis
MH  - Humans
MH  - Infant
MH  - Reticulocytes/cytology
MH  - Thalassemia/genetics/*physiopathology
EDAT- 1987/08/01 00:00
MHDA- 1987/08/01 00:01
CRDT- 1987/08/01 00:00
PHST- 1987/08/01 00:00 [pubmed]
PHST- 1987/08/01 00:01 [medline]
PHST- 1987/08/01 00:00 [entrez]
AID - 10.1002/ajh.2830250405 [doi]
PST - ppublish
SO  - Am J Hematol. 1987 Aug;25(4):389-400. doi: 10.1002/ajh.2830250405.