Acute Leukaemias

(Normal WCC 4-11 x 10⁹/l) - in leukaemia can be raised to 400-500 x 10⁹/l

• defined as >30% blasts in bone marrow

• untreated can be fatal in 6 weeks

• usually better cure rates than chronic leukaemias

Types

• Lymphoid / lymphoblastic - B or T cell lineage

• Myeloid - everything else

Biology

• immature cells / blasts

  • large nucleus

• divide rapidly

• starts in bone marrow but can be in liver, spleen, lymph nodes

Clinical Features

• Cytopenias

  • anaemia

  • neutropenia

    • bacterial or fungal infections

  • thrombocytopenia

    • bleeding

    • easy bruising

    • purpura

• Neurological signs

  • due to CNS infiltration (common in ALL)

• Testicular infiltration

• Hypopyon

Diagnosis

• clinical features

• Full blood count and blood film

• bone marrow aspirate and trephine

  • morphology

    • Auer rods pathognomic for AML (M1-M3 types)

• Immunophenotyping

• Cytogenetic and molecular analysis

  • e.g. for Philadelphia chromosome, t(9,22) poor prognostic predictor in ALL/CML

Classification

• ALL into L1 to L3 (FAB) or  B / T

• AML into M0 to M7

  • M3 / promyelocytic

Chromosomal abnormalities

• ALL

  • poor prognosis

    • t(4,11)

    • t(6,22)

    • t(1,19)

  • good prognosis

    • t(12, 21)

• AML

  • poor prognosis

    • 5q deletion

    • 7q deletion

    • complex

  • good prognosis

    • t(15,17) - if they don't die from bleeding first

    • t(8,21)

    • inv 16

Possible causes of leukaemia

• viruses

  • Epstein-Barr (EBV)

  • HTLV-1

• radiation

• benzene

• chromosomal abnormalities

  • e.g. Down's syndrome

• ALL peaks at 4-5 years old

Treatment

• Supportive care

  • blood products

  • protection against infection

• Chemotherapy

Prognosis

• 80% children with ALL cured

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