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
Presentation:
- 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:
- monitoring only if asymptomatic
- chlorambucil to decrease lymphocyte count, improve marrow function and decrease node size
- 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.
