24.CLL/SLL

Question 1

What is CLL/SLL?

Answer 1

monoclonal accumulation of functionally incompetent mature B-lymphocytes

Question 2

aggressive transformation of CLL/SLL into high-grade lymphoma (usually DLBCL) is called?

Answer 2

Richter’s syndrome

Question 3

what immunologic abnormalities can patients with typical B-cell CLL present with?

Answer 3

AHA
autoimmune thrombocytopenia
hypogammaglobulinemia (↑ susceptibility to infections)

Question 4

what markers are used to asses prognosis in CLL?

Answer 4

1. ZAP-70 (Expressed by T-cells)–> poor prognosis
2. CD38 –> poor prognosis
3. immunoglobulin variable region-> patients with mutated Ig variable region respond better and have better survival

Question 5

what cytogenetics have unfavorable prognosis in CLL?

Answer 5

11q22-23 and 17p13

Question 6

what cytogenetics have a favorable prognosis in CLL?

Answer 6

13q14

Question 7

what system staging is used in CLL?

Answer 7

Rai stage (0-IV)
Binet group (A-C)

Question 8

treatment options for CLL

Answer 8

FCR combination: Fludarabine, cyclophosphamide, Rituximab

• chlorambucil (elderly)
• Alemtuzumab (anti-CD52)
• Ibrutinib (Bruton TKI)
• Venetoclax (BCL-2 inhibitor)