Clonal evolution of aplastic anaemia to myelodysplasia/acute myeloid leukaemia and paroxysmal nocturnal haemoglobinuria

Leuk Lymphoma. 1999 Apr;33(3-4):231-41. doi: 10.3109/10428199909058423.

Abstract

Aplastic anaemia (AA) is a non-malignant haemopoietic disorder characterised by peripheral blood pancytopenia and a hypocellular bone marrow. Successful management of acquired AA including treatment with immunosuppressive agents, mainly antithymocyte globulin (ATG) and cyclosporin or allogeneic haemopoietic stem cell transplantation, has resulted in long-term survival of many patients. The later evolution of complicating clonal disorders such as paroxysmal nocturnal haemoglobinuria, myelodysplasia and acute myeloid leukaemia in patients treated with immunosuppressive therapy may be a manifestation of the natural history of the aplasia, the development of which may or may not be increased by immunosuppressive therapy. A persistent, profound deficiency and/or defect in the stem cell compartment, despite haematological recovery after immunosuppressive therapy, may create an unstable situation which predisposes to later clonal disorders. A review of the progression of AA to clonal disorders is now outlined.

Publication types

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

MeSH terms

  • Acute Disease
  • Anemia, Aplastic / blood
  • Anemia, Aplastic / physiopathology*
  • Anemia, Aplastic / therapy
  • Antilymphocyte Serum / therapeutic use
  • Bone Marrow / pathology
  • Granulocyte Colony-Stimulating Factor / adverse effects
  • Hemoglobinuria, Paroxysmal / blood
  • Hemoglobinuria, Paroxysmal / physiopathology*
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Leukemia, Myeloid / blood
  • Leukemia, Myeloid / physiopathology*
  • Myelodysplastic Syndromes / blood
  • Myelodysplastic Syndromes / physiopathology*
  • Pancytopenia

Substances

  • Antilymphocyte Serum
  • Immunosuppressive Agents
  • Granulocyte Colony-Stimulating Factor