An aberrant type of congenital dyserythropoietic anemia associated with a beta-thalassemia trait

Ann Hematol. 1991 May;62(5):184-7. doi: 10.1007/BF01703146.

Abstract

A child is described who suffered from a severe congenital anemia. The anemia persisted and a regular transfusional regimen became necessary. Bone marrow aspirates showed an erythroblastic hyperplasia associated with some dyserythropoietic features. A most striking and constant finding was the presence of many inter-erythroblastic chromatin bridges. The reticulocyte count was always low, in spite of the increased erythropoietic activity. A beta-thalassemia trait inherited from the mother was demonstrated. The hypothesis of dyserythropoiesis/thalassemia syndrome was put forward, based on the assumption that the two genes may have interacted with each other.

Publication types

  • Case Reports

MeSH terms

  • Anemia, Dyserythropoietic, Congenital / complications*
  • Anemia, Dyserythropoietic, Congenital / pathology
  • Anemia, Dyserythropoietic, Congenital / therapy
  • Blood Transfusion
  • Bone Marrow / pathology
  • Chromatin / pathology
  • Erythroblasts / ultrastructure
  • Humans
  • Hyperplasia
  • Infant
  • Male
  • Splenectomy
  • Thalassemia / complications*
  • Thalassemia / pathology
  • Thalassemia / therapy

Substances

  • Chromatin