Wednesday, 15 September 2010

MRCP revision battle 12.2: CLL

I keep clerking patients who say they've got 'CLL, doctor' and I nod sagely and scribble it down, while desperately trying to recall any of my med school haematology lectures.  My brief summary for MRCP means that now hopefully my sage nod is slightly more appropriate!!

CLL (=chronic lymphocytci leukaemia) is a monoclonal proliferation of mature lymphocytes.

99% of cases of CLL involve B cells.

CLL is important clinically as it is the commonest cause of lymphocytosis in the elderly.
Males are more often affected than females, 2:1

  • no symptoms in 25%
  • recurrent infections (due to hypogammaglobulinaemia)
  • anaemia (warm AIHA in 10-15%)
  • decreased weight
  • sweats
  • anorexia

Signs includer:
  • enlarged rubbery, non-tender nodes
  • hepatomegaly
  • splenomegaly

Blood tests will show:
  • raised lymphocytes
  • low Hb, neutrophils and platelets
  • smear/smudge cells on blood film

Staging is done by the Rai classification, which can also be used to give the prognosis:
  • Stage 0 - lymphocytosis alone - mean survival >13 yrs
  • Stage 1 - lymphocytosis plus lymphadenopathy - mean survival 8 yrs
  • Stage 2 - lymphocytosis plus spleno/hepato megaly - mean survival 5 yrs
  • Stage 3 - lymphocytosis plus anaemia (defined as Hb <11) - mean survival 2 yrs
  • Stage 4 - lymphocytosis plus platelets <100 - mean survival 1 yr

Indicators of poorer prognosis include:
  • being male
  • age >70
  • lymphocytes >50
  • prolymphocytes >10%
  • doubling rate <12 months
  • raised LDH
  • CD 38 positive
  • ZAP 70 + (if ZAP 70 -ive, mean survival 25 years)

Management of CLL is:
  1. monitoring only if asymptomatic
  2. chlorambucil to decrease lymphocyte count, improve marrow function and decrease node size
  3. fludarabine (a chemo agent, purine analog that works by inhibiting DNA synthesis) if:
    • bulky disease
    • cytopenias due to marrow failure
    • short lymphocyte doubling time

CLL may transform to Richters syndrome.
Richters syndrome is the transformation of CLL to agressive B cell lymphoma.  LDH is raised.

Now onwards to a subtype of CLL, hairy-cell leukaemia.