ABC of clinical haematology: The acute leukaemias
BMJ 1997; 314 doi: https://doi.org/10.1136/bmj.314.7082.733 (Published 08 March 1997) Cite this as: BMJ 1997;314:733- R J Liesner,
- A H Goldstone
Acute leukaemia is a clonal (that is, derived from a single cell) malignant disorder affecting all age groups from infancy to old age. It is characterised by the accumulation of abnormal white blood cells in the bone marrow which replace normal marrow tissue, including haemopoietic precursor cells. This results in bone marrow failure and peripheral blood involvement. Infiltration of various organs is also a feature of some forms of leukaemia.
Aetiological factors in acute leukaemia
Unknown (usually)
Hereditary
Down's syndrome
Bloom's syndrome
Fanconi's anaemia
Ataxia telangiectasia
Kleinfelter's syndrome
Osteogenesis imperfecta
Wiskott-Aldrich syndrome
Leukaemia in siblings
Chemicals
Chronic benzene exposure
Alkylating agents (chlorambucil, melphalan)
Radiation
Predisposing haematological diseases (myeloproliferative disorders, myelodysplasia, and aplastic anaemia).
Viruses (HTLV-I causing adult T cell leukaemia/lymphoma)
In most cases the aetiology is not obvious, but some constitutional and acquired disorders do predispose to acute leukaemia.
In the past 40 years advances in the treatment of acute leukaemia have improved the chance of cure from virtually zero to 20-75%, depending on age and type of leukaemia. This has largely been the result of clinical trials—many of which are still ongoing—and the development and continued improvements in bone marrow transplantation.
Classification
Acute leukaemia is subdivided into (a) acute lymphoblastic leukaemia, in which the abnormal proliferation is in the lymphoid progenitor cells (that is, immature lymphocytes) and (b) acute myeloid leukaemia, which involves the myeloid lineages (that is, cells from which neutrophils, eosinophils, monocytes, basophils, megakaryocytes, etc are derived). The distinction between the two leukaemias is based on morphological, cytochemical, and immunological differences and is of paramount importance as the treatment and prognosis are usually different.
FAB* classification of acute myeloid leukaemia
M0 Acute myeloid leukaemia with minimal evidence of myeloid differentiation
M1 Acute myeloblastic leukaemia without maturation
M2 Acute myeloblastic leukaemia with maturation
M3 Acute promyelocytic leukaemia
M4 Acute myelomonocytic leukaemia
M5 Acute …
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