Keypoint blood. 20.12.01


     DOWN'S SYNDROME: BLOOD DISORDERS / LEUKAEMIA
                                                                      Key Points

(based on conference paper by Professor Judith Chessells at RSM
conference. April 2001)

·  Over 60% of neonates polycythaemic ­ unrelated to
      congenital heart disease

·  MCV increased at all ages

·  Almost unique risk of transient abnormal myelopoesis (TAM)
      in newborns (approx 10%)

          · Morphologically indistinguishable from megakaryocytic leukaemia
          · Usually asymptomatic
          · Usually resolves spontaneousely without treatment
          · Around 25% with TAM may develop acute myeloid leukaemia (AML) later
                 in childhood

·  Childhood leukaemia 20 times more frequent than in other
      children. Risk around 1/100. Both AML and ALL (acute
      lymphoblastic leukaemia). AML is usually megakaryoblastic
      (very rare in other children ).

·  Children should be entered in national leukaemia trials and
      should usually be treated according to protocol.

·  Response to treatment of both AML and ALL is good. 5 year
      survival for AML in the UK around 60% ( same as other
      children). For ALL around 75% (poorer than other children).

·  Increased risk of death whilst on intensive therapy both
      during induction and further treatment,  because of inherent
      increased susceptibility to infection. Extreme vigilance and
      maximum supportive care is necessary at these times.

·  Relapse rate less than for other children- survival difference
      is largely due to increased risk of death during treatment

·  Peak age at onset of leukaemia is before 4 years. No cases
      recorded after age 29. Low risk of other childhood cancers.

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DSMIG(UK) 2001.     www.dsmig.org.uk                                                                                                                         14.01.02