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Effects of thalassemia and microcytosis on the hematologic and
vasoocclusive severity of sickle cell anemia
MH Steinberg, W Rosenstock, MB Coleman, JG Adams, O Platica, M Cedeno, RF Rieder, JT Wilson, P Milner and S West
The characteristic clinical heterogeneity of sickle cell anemia (HbSS) may
be, in part, a result of its interactions with alpha-thalassemia. Although
alpha-thalassemia clearly affects some hematologic features of HbSS, its
role in modulating the vasoocclusive severity of disease is not clear. To
further explore this relationship, we examined the incidence of painful
episodes, acute chest syndrome, aseptic bone necrosis, and leg ulcers in 3
patient groups with sickle cell disease: (1) 2,147 patients over age 2 yr,
stratified according to mean corpuscular volume (MCV); (2) 183 patients
selected on the basis of microcytosis and elevated HbA2, on whom globin
biosynthesis studies were done; and (3) 125 patients who had alpha-globin
genotype assigned by restriction endonuclease gene mapping. When patients
were stratified by MCV, there was a reciprocal relationship between HbA2
levels and MCV, reflecting the presence of patients with beta o and alpha-
thalassemia in the low MCV groups. The erythrocyte indices and HbA2 levels
in patients classified as HbSS-alpha-thalassemia, by either globin
synthesis studies or gene mapping, were very similar to those previously
reported by others. Neither microcytosis, beta o, or alpha- thalassemia
appeared to provide any clear protection from the vasoocclusive
complication evaluated, and the prevalence of aseptic necrosis was
increased in patients with microcytosis over age 20 yr and in groups with
alpha-thalassemia. The effects of a reduced MCV and mean corpuscular
hemoglobin concentration (MCHC), of possible benefit by themselves, when
accompanied by a reduction in hemolysis and rise in hemoglobin
concentration, as in HbSS-alpha-thalassemia, may cause sufficient rise in
blood viscosity in critical vascular beds to impair blood flow and negate
any amelioration of vasoocclusive complications in HbSS.
Volume 63,
Issue 6,
pp. 1353-1360,
06/01/1984
Copyright © 1984 by The American Society of Hematology
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