• uncommon

• 10/100 000 / year

• Acute or chronic

• myeloid or lymphoid

• ≥ 1/2 present acutely (ALL, AML

• ALL - childhood

• CLL - elderly

• myelodysplastic syndromes = preleukaemia

• diagnosis:

  • blood film

  • bone marrow

• aetiology

  • radiation

    • ALL, AML, CML

  • Benzene

    • AML

  • Gnetic

    • Fanconi anaemia

    • risk elevated 30x in people with trisomy 21

  • Viruses

Acute leukaemia

• increase in incidence with advancing age

  • ALL > in younger people

• majority present with sympotms reflecting inadequate haematopoiesis 2ary to leukaemic cells infiltration of marrow

  • anaemia

  • leucopenia - recurrent infections

  • thrombocytopenia

    • bleeding/bruising

    • especially acute promyelocytic leukaemia

  • bone pain - marrow infiltration

• exam - may be unremarkable

  • pallow

  • fever

  • petechiae, fundal haemorrhoage

  • lymphadenopathy

  • hepatosplenomegaly (especially ALL)

  • skin lesions (AM(mono)L

  • testicular enlargement (ALL)

  • cranial nerve palsy - ALL

• investigation:

  • blood count

    • hb low

    • wcc usually raised (sometimes low)

    • blast cells

  • bone marorw aspirate

    • increase cellularity

    • decrease erythropoeitis

    • decrease megakaryocytes

    • trilineage dysplasia

    • confirm by FISH

  • CXR

    • widened mediastinum

      • T lymphoblastic leukaemia

    • CSF

  • Management

    • Palliative

    • Curative

    • Supportive

      • Avoidance of aneamia

      • prevention fo bleeding

      • Treatment of infection

        • prophylactically

        • therpeutically

      • control of hyperuraecaemia

        • hydration

        • prophylactic allopurinol

        • occasionally rasburicase

• most recurrance within 3 years

  • outcome extremely poor

Acute myeloid leukameia

• treatment with curative intent majority \<60yo

Acute lymphoblastic leukaemia

• commoneest malignancy in childhood

• Need CNS directed therapy

  • intrathecal chemotherapy

  • as soon as blasts are cleared from blood

• Prognostic factors

  • age

  • WCC @ time of diagnosis

  • Immunophenotype

  • Cytogenetic abberations

  • time to response

  • minimal residual disease

• prognosis in childhood = excellant

  • complete remission in almost all

  • 80% alive without recrrence at 5 years

Chronic leukaemia

Chronic myeloid leukaemia

• 14% of leukaemia

• disease of adults

  • 40-60yo = peak

• associated with philidephia chromosone in 97% of cases

  • also seen in ALL

  • t(9;22)(q34;q11)

• slowly progressive course

• if not initially ured will be followed evenutally by “blast crisis or melofbrosis and death after 3-4yrs

• usually presents in chronic phase

  • symptoms:

    • anaemia

    • abdominal discomfort - splenomegaly

    • weigt loss

    • fevers, sweats

    • headache (oss.)

    • bruising

    • priaprism

    • lymphadnopathy - sugggest blast crisis

Chronic lymphocytic leukaemia

• commonest leukameia

• later life

• B-cell (95%)

• majority asymptomatic

  • symptoms

    • recurrent infection

    • anaemia

    • painless lymphadenopathy

    • left upper quadrant discomfort (splenomegaly)

      • often massive hepatosplenomegaly

• Smudge cells

• indications for treatment:

  • marrow failure manifest by worsening anaemai and/or thrombocytopenia

  • recurrent infection

  • massive/progressive splenomegaly or lymphadenopathy

  • preogressive disease maifest by doubling of lumphocyte count in 6/12

  • systemic features

  • presence of heamolysis or other immune mediated cytopenias

• management

  • anaemia 2ary haemolysis -> steroids

    • splenectomy

  • Allopurinol -> hyperuricaemia

• may undergo lymphomatous (Richter’s) transformation in 5-10% of cases

  • diffuse large B-cell lymphoma