Ringed sideroblasts in β-thalassemia

Pediatr Blood Cancer. 2017 May;64(5):10.1002/pbc.26324. doi: 10.1002/pbc.26324. Epub 2016 Nov 3.

Abstract

Symptomatic β-thalassemia is one of the globally most common inherited disorders. The initial clinical presentation is variable. Although common hematological analyses are typically sufficient to diagnose the disease, sometimes the diagnosis can be more challenging. We describe a series of patients with β-thalassemia whose diagnosis was delayed, required bone marrow examination in one affected member of each family, and revealed ringed sideroblasts, highlighting the association of this morphological finding with these disorders. Thus, in the absence of characteristic congenital sideroblastic mutations or causes of acquired sideroblastic anemia, the presence of ringed sideroblasts should raise the suspicion of β-thalassemia.

Keywords: ringed sideroblasts; sideroblastic anemia; thalassemia.

Publication types

  • Research Support, N.I.H., Extramural

MeSH terms

  • Adolescent
  • Adult
  • Anemia, Sideroblastic / diagnosis
  • Anemia, Sideroblastic / pathology*
  • Bone Marrow Cells / cytology
  • Bone Marrow Cells / pathology*
  • Bone Marrow Examination
  • Child
  • Erythroblasts / cytology
  • Erythroblasts / pathology*
  • Erythrocytes, Abnormal
  • Female
  • Hematologic Diseases / complications
  • Humans
  • Infant
  • Male
  • beta-Thalassemia / diagnosis
  • beta-Thalassemia / pathology*

Supplementary concepts

  • Macrocytosis, Familial