Diamond-Blackfan Anaemia

  • Congenital hypoplastic anaemia
  • Normocytic anaemia with Hb ~ 3-4g
  • Normal platelets & wbc

Associations

  • Thumb anomalies
  • Radial hemimelia
  • Klippel Feil
  • Sprengels
  • Hypoplasia  of the first ray
  • Cleft palate
  • Small Jaw
  • Renal Anomalies

Presentation

  • Clinical presentation is at 3-6 months with listless, irritable child

Treatment

  • Steroids
  • Transfusion