The clinical severity of hemoglobin S/Black (A γδβ)0 -thalassemia.

Journal Article (Journal Article)

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.

Full Text

Duke Authors

Cited Authors

  • Cancio, MI; Aygun, B; Chui, DHK; Rothman, JA; Scott, JP; Estepp, JH; Hankins, JS

Published Date

  • November 2017

Published In

Volume / Issue

  • 64 / 11

PubMed ID

  • 28453928

Pubmed Central ID

  • PMC6615052

Electronic International Standard Serial Number (EISSN)

  • 1545-5017

Digital Object Identifier (DOI)

  • 10.1002/pbc.26596


  • eng

Conference Location

  • United States