Chronic Lymphocytic Leukaemia

Question 1

Definition of chronic lymphocytic leukaemia

Answer 1

Caused by a monoclonal proliferation, and hence accumulation, of functionally incompetent lymphocytes (B Cells)
• Predominantly found in peripheral blood
• Lymphocytosis is sustained for at least 3 months

Question 2

Aetiology of chronic lymphocytic leukaemia

Answer 2

• It is the COMMONEST leukaemia in adults

• Exact cause is unclear, likely develops due to accumulation of mutations affecting oncogenes and tumour suppressor genes
• Trisomies and deletions influence risk

Question 3

Pathophysiology of chronic lymphocytic leukaemia

Answer 3

• The chronic lymphocytic leukaemia cells can infiltrate the lymphatic system and haematopoietic organs such as liver, spleen and bone marrow
◦ Can hence result in Hepatosplenomegaly, lymphadenopathy etc
• The dysfunctional lymphocytes would increase risk of recurrent infections

Question 4

History and examination of chronic lymphocytic leukaemia

Answer 4

• Often asymptomatic: lymphocytosis picked up incidentally
• SOB and fatigue: likely due to anaemia
• Painless lymphadenopathy
• Hepatosplenomegaly: splenomegaly more common
• B symptoms: fever, weight loss, night sweats
• Recurrent infections
• Petechiae: due to dysfunctional lymphocytes and subsequent immunoglobulin deficiency

• Age is risk factor

Question 5

Investigations for chronic lymphocytic leukaemia

Answer 5

• WBC count: Elevated lymphocytes needed for diagnosis (persists for at least 3 months)
• Blood film: smudge/smear cells present
• FBC: Hb and platelets may be reduced due to marrow infiltration
• Flow cytometry: Identify markers found on monoclonal B lymphocytes

Question 6

Treatment of chronic lymphocytic leukaemia

Answer 6

Asymptomatic early stage:
1) observation: Do not need immediate treatment. Active surveillance recommended (every 3 months check FBC, flow cytometry etc)

Symptomatic or advanced disease:
1) Targeted therapy or chemoimmunotherapy: can take acalabrutinib

Question 7

Complications of chronic lymphocytic leukaemia

Answer 7

• Autoimmune haemolytic anaemia:
• Hypogammaglobulinaemia: due to the dysfunctional lymphocytes not being able to produce antibodies, increases risk of infections further
• Richter transformation: when leukaemia enters lymph node and becomes Non-Hodgkin lymphoma. Aggressive

Question 8

Prognosis of chronic lymphocytic leukaemia

Answer 8

Death is often due to infection or due to Richter transformation
1/3 never progress
1/3 progress slowly
1/3 progress actively