Introduction

Aplastic is a rare condition characterised by pancytopenia and a hypoplastic bone marrow.

Aetiology

  • Idiopathic
  • Congenital: Fanconi anaemia, dyskeratosis congenita
  • Drugs: cytotoxics, chloramphenicol, sulphonamides, phenytoin, gold
  • Toxins: benzene
  • Infections: parvovirus, hepatitis
  • Radiation

Clinical features

  • Normochromic, normocytic anaemia
  • Leukopenia, with lymphocytes relatively spared
  • Thrombocytopenia
  • May be the presenting feature acute lymphoblastic or myeloid leukaemia
  • A minority of patients later develop paroxysmal nocturnal haemoglobinuria or myelodysplasia

Management

Supportive
  • Blood products
  • Prevention and treatment of infection

Anti-thymocyte globulin (ATG) and anti-lymphocyte globulin (ALG)
  • Prepared in animals (e.g. rabbits or horses) by injecting human lymphocytes
  • Is highly allergenic and may cause serum sickness (fever, rash, arthralgia), therefore steroid cover usually given
  • Immunosuppression using agents such as ciclosporin may also be given

Stem cell transplantation
  • Allogeneic transplants have a success rate of up to 80%