The clinical severity of hemoglobin S/Black (A γδβ)0 -thalassemia. Pediatr Blood Cancer 2017 Nov;64(11)
Date
04/30/2017Pubmed ID
28453928Pubmed Central ID
PMC6615052DOI
10.1002/pbc.26596Scopus ID
2-s2.0-85018314607 (requires institutional sign-in at Scopus site) 4 CitationsAbstract
Hemoglobin S/Black (A γδβ)0 -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, including vaso-occlusive crisis, acute chest syndrome, avascular necrosis, and cholelithiasis. On the basis of these findings, we recommend that patients with this rare disorder receive specialized hematology care according to SCD guidelines.
Author List
Cancio MI, Aygun B, Chui DHK, Rothman JA, Scott JP, Estepp JH, Hankins JSMESH terms used to index this publication - Major topics in bold
AdolescentAdult
Anemia, Sickle Cell
Child
Child, Preschool
Female
Fetal Hemoglobin
Hemoglobin, Sickle
Humans
Infant
Infant, Newborn
Male
Prognosis
Severity of Illness Index
Thalassemia
Young Adult