Chronic Lymphocytic Leukaemia

Commonest haematological malignancy
  • 90% are over 50
  • very rare under 25

Clinical Features

  • 25-30% asymptomatic
  • Fatigue
  • Malaise
  • Weight loss
  • Infections
  • bleeding
  • anaemia
  • lymphadenopathy
  • organomegaly (liver, spleen)

Investigations

  • Full blood count (FBC)
    • lymphocytes >5 x 109/L
    • 2 occasions
  • Blood film
    • smear cells
  • bone marrow aspirate / trephine
  • autoimmune phenomena
    • reticulocytes
    • direct Coombes test
  • Immunophenotyping
  • Cytogenetics
  • Bilirubin
  • LDH
  • Immunoglobulin levels
    • may be low
  • Protein electrophoresis
    • shows paraprotein

Immunophenotyping

  • 95% B-CLL
    • Express CD19,
    • CD20,
    • surface immunoglobulin,
    • CD5 (aberrantly expressed by leukaemic cells)
  • 5% T-CLL
    • CD2/3
    • CD 4
    • CD 8

Aetiology

  • unknown
  • some familial
  • race distribution
  • association with chromosomal abnormalities
    • Trisomy 21 17%
    • Chr 13 - 13%
    • Chr 14

Staging

Rai

  • 0 - Blood and bone marrow lymphocytes
  • 1 - Lymphocytosis and lymphadenopathy
  • 2 - Lymphocytosis / lymphadenopathy and organomegaly
  • 3 - anaemia (Hb <11g/dL)
  • 4 - Thrombocytopenia (Platelets <100 x 109/L

Binet

  • A - Lymphocytosis with =< 3 lymph node areas
  • B - > 3 lymph node areas
  • C - anaemia / thrombocytopenia

Treatment

Indications

(might not need treating because old population, long survival, toxicity of chemotherapy)

  • BM failure
    • anaemia
    • thrombocytopenia
  • Symptomatic disease
  • Bulky disease
  • Progressive disease
    • short doubling time
    • Transformation to prolymphocytic leukaemia
    • Richter's (CLL transforms to High grade non-Hodgkins lymphoma)

Supportive Care

  • Transfusions
  • Antibiotics / Vaccines
  • IV immunoglobulins

Chemotherapy

  • Steroids
    • especially if autoimmune problems
  • alkylating agents
  • combination chemotherapy
  • purine analogues
    • fludarabine
    • DCF
    • 2CDA
  • Monoclonal antibodies
    • Campath-1H
    • Rituximab

Bone Marrow Transplant / Stem cell transplant

  • Experimental

Prognosis

Factors

  • Stage
  • Lymphocyte doubling time
    • Good > 12 months
    • Bad if <12 months
  • Bone marrow histology
  • T-CLL has worse prognosis
  • Karyotype

Survival

  • Stage 0 >150 months
  • Stage 3/4 19 months
 

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