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leukaemia

acute lymphoblastic leukaemia
1. =80% of leukaemia in kids
2. clinical presentation
  1. peaks @ 2-5 years
  2. results from disseminated disease & systemic ill health from infiltration of bone marrow or other organs w/ leukaemic blast cells
    1. general
      1. malaise
      2. anorexia
    2. bone marrow infiltration
      1. anaemia
        pallor, lethargy
      2. neutropenia
        infection
      3. thrombocytopenia
        bruising, petechiae, nose bleeds
      4. bone pain
    3. reticulo-endothelial infiltration
      1. hepatosplenomegaly
      2. lymphadenopathy, superior mediastinal obstruction (uncommon)
    4. other organ infiltration (rare @ diag, more often @ relapse)
      1. CNS
        headaches
        vomiting
        nerve palsies
      2. testes
        testicular enlargement
  3. in most kids, ALL presents insidiously over several weeks
    1. in some presentation& progress is rapid
3. Ix
  1. abnormal FBC in most
    1. low Hb
    2. thrombocytopenia
    3. evidence of circulating leukaemic blast cells
  2. bone marrow exam
    1. to confirm diag
    2. to ID immunological & cytogenetic characteristics that give prognostic info
  3. CXR
    1. to ID mediastinal mass
      1. characteristic of T cell disease
  4. morphological classification
    1. for ALL & AML
    2. + immunological phenotyping to further subclassify ALL
      1. common subtype (75%)
      2. T cell subtype (15%)
4. Mx
  1. prognostic factors (& also indicate intensity of therapy)
    1. age
      1. high risk features
        <1 year or > 10 years old
    2. tumour load (measured by WBC)
      1. high risk features
        > 50 x10^9/L
    3. cytogenetic/molecular genetic abnormalities in tumour cells
      1. high risk features
        e.g. MLL rearrangement, t(4:11), hypodiploidy (<44 chromosomes)
    4. speed of response to initial chemo
      1. high risk features
        persistence of leukaemic blasts in bone marrow
    5. minimal residual disease (submicroscopic levels of leukaemia detected by PCR)
      1. high risk feature
        high
  2. remission induction
    1. before Rx anaemia may need correcting
      1. blood transfusion
    2. minimise risk of bleeding
      1. platelet transfusion
    3. treat infection
    4. additional hydration & allopurinol (or urate oxidase when WCC high & risk greater)
      1. to protect renal function vs effects of rapid cell lysis
    5. remission implies eradication of leukaemic blasts & restoration of normal marrow function
    6. 4 weeks of combo chemo given
      1. current schedules reach remission rates of 95%
  3. intensification
    1. block of chemo given to consolidate remission
    2. improves cure rates but w/ increased toxicity
  4. central nervous sys
    1. cytotoxic drugs penetrate poorly into CNS
    2. additional Rx w/ intrathecal chemo
      1. to prevent CNS relapse
      2. as leukaemic cells in CNS may survive effective systemic Rx
  5. continuing therapy
    1. moderate intensity chemo continued up to 3 years from diag
    2. routine co-trimoxazole prophylaxis
      1. to prevent Pneumocystitis carinii pneumonia
  6. treatment of relapse
    1. high dose chemo, often + total body irradiation + bone marrow transplantation
      1. alternative to chemo after a relapse
acute myeloid leukaemia
acute non-lymphocytic leukaemia
chronic myeloid leukaemia & other myeloproliferative disorders
1. rare

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leukaemia

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	Created by v.djabatey almost 11 years ago