The clinical severity of hemoglobin S/Black (A ???)0 -thalassemia.
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ABSTRACT:
SUBMITTER: Cancio MI
PROVIDER: S-EPMC6615052 | biostudies-literature | 2017 Nov
REPOSITORIES: biostudies-literature
Publications
The clinical severity of hemoglobin S/Black (<sup>A</sup> γδβ)<sup>0</sup> -thalassemia.
Cancio Maria I MI Aygun Banu B Chui David H K DHK Rothman Jennifer A JA Scott J Paul JP Estepp Jeremie H JH Hankins Jane S JS
Pediatric blood & cancer 20170428 11
Hemoglobin S/Black (<sup>A</sup> γδβ)<sup>0</sup> -thalassemia is a rare sickle cell disease (SCD) variant. On the basis of limited descriptions in the literature, the disease is reported as a mild microcytic anemia with an uncomplicated course. We report the clinical and laboratory data of nine patients whose diagnoses were confirmed by DNA-based techniques. Despite having mild anemia and high fetal hemoglobin level postinfancy, these patients developed many of the classic complications of SCD, ...[more]
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