Aplastic Anaemia

[ Home ] [ Up ]

Pages Below:

Aplastic Anaemia

  • pancytopenia with hypocellularity of the bone marrow
  • no leukaemic, cancerous or other abnormal cells in peripheral blood / bone marrow

Causes

Primary

  • congenital
    • Fanconi's Anaemia
  • Idiopathic Acquired (67%)

Secondary

  • chemicals
    • benzene
  • drugs
    • chemotherapeutic
    • idiosyncratic
  • insecticides
  • ionising radiation
  • infections
    • viral
      • hepatitis
      • EBV
        HIV
        parvovirus
    • other
      • TB
  • paroxysmal nocturnal haemoglobinuria

Clinical Features

  • anaemia
  • bleeding
  • infection

Investigation

  • FBC
    • pancytopenia
    • virtual absence of reticulocytes
  • Bone Marrow Trephine
    • reduced cellularity
    • increased fat spaces

Treatment

  • avoid infection
    • broad-spectrum IV antibiotics if suspected
  • supportive care
  • eliminate cause
  • consider bone marrow transplantation
    • <50 + sibling donor 75-90% long-term survival
  • immmunsupression
    • anti-lymphocyte globulin (haematoloigcal recovery 50-60%)
    • ciclosporin (80% recovery)
  • androgens
  • steroids
    • relieve serum sickness of ALG
    • in Diamond-Blackfan syndrome (congenital pure red cell aplasia)
  • thymectomy

Prognosis

  • poor if
    • PMN <0.5 x 109
    • Platelets <20 x 109
    • Reticulocyte count <40 x 109
  •  
 

[ Home ] [ Up ]