CLL

Question 1

What is the most common leukemia seen ?

Answer 1

most common form of leukaemia seen in adults.

Question 2

What type of cell is CLL predominantly made out of ?

Answer 2

monoclonal proliferation of well-differentiated lymphocytes which are almost always B-cells

Question 3

What are some of the clinical features of CLL?

Answer 3

Lymphadenopathy more than CML

weight loss

Question 4

Investigation of CLL

Answer 4

full blood count:

lymphocytosis

anaemia: may occur either due to bone marrow replacement on autoimmune hemolytic anaemia (AIHA)

thrombocytopenia: may occur either due to bone marrow replacement on immune thrombocytopenia (AIHA)

blood film: smudge cells (also known as smear cells)

immunophenotyping is the key investigation
most cases can be identified using a panel of antibodies specific for CD5, CD19, CD20 and CD23

Question 5

What type of AIHA is most commonly associated with CLL?

Answer 5

Warm autoimmune haemolytic anaemia occurs in around 10-15%

Question 6

Indications for treatment of CLL?

Answer 6

worsening of anaemia and/or thrombocytopenia

massive (>10 cm) or progressive lymphadenopathy

massive (>6 cm) or progressive splenomegaly

progressive lymphocytosis: > 50% increase over 2 months or lymphocyte doubling time < 6 months

systemic symptoms: weight loss > 10% in previous 6 months,

fever >38ºC for > 2 weeks, extreme fatigue, night sweats

autoimmune cytopaenias e.g. ITP

Question 7

Management

Answer 7

patients who have no indications for treatment are monitored with regular blood counts

fludarabine, cyclophosphamide and rituximab (FCR) has now emerged as initial treatment of choice for the majority of patients
ibrutinib may be used in patients who have failed a previous therapy

Question 8

poor prognostic factors of CLL ?

Answer 8

deletions of part of the short arm of chromosome 17 (del 17p) are seen in around 5-10% of patients and are associated with a poor prognosis

CD38 expression positive

TP53 mutation

male sex

age > 70 years

lymphocyte count > 50

prolymphocytes comprising more than 10% of blood lymphocytes

lymphocyte doubling time < 12 months

raised LDH

CD38 expression positive

TP53 mutation

Question 9

good prognosis of CLL

Answer 9

deletion of the long arm of chromosome 13 (del 13q) - most common abnormality, being seen in around 50% of patients. associated with a good prognosis