Successful hematopoietic stem cell transplantation in a patient with congenital dyserythropoietic anemia type II

Pediatr Transplant. 2014 Jun;18(4):E130-3. doi: 10.1111/petr.12254. Epub 2014 Apr 12.

Abstract

CDA are a group of inherited, rare diseases that are characterized by dyserythropoiesis and ineffective erythropoiesis associated with transfusion dependency in approximately 10% of cases. For these latter patients, the only curative treatment is HSCT. There are very limited data on HSCT experience in this rare disease. Herein, we report a five-yr six-month-old girl with compound heterozygous mutations in SEC23B gene, who was diagnosed to have CDA type II and underwent successful HSCT from her matched sibling donor.

Keywords: SEC23B; congenital dyserythropoietic anemias; iron; transplantation.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Anemia, Dyserythropoietic, Congenital / genetics
  • Anemia, Dyserythropoietic, Congenital / therapy*
  • Child, Preschool
  • Female
  • Genetic Markers
  • Hematopoietic Stem Cell Transplantation*
  • Heterozygote
  • Humans
  • Mutation
  • Siblings
  • Vesicular Transport Proteins / genetics

Substances

  • Genetic Markers
  • SEC23B protein, human
  • Vesicular Transport Proteins