Lymphoma

Question 1

What is the definition of lymphoma?

Answer 1

Group of diseases caused by malignant lymphocytes (B& T) that accumulate in lymph nodes to form rumours.
The lymphocytes undergo uncontrolled proliferation & are anti-apoptotic.

Question 2

What are characteristic cells in Hodgkin’s lymphoma?

Answer 2

Presence of Reed-Sternberg cells in tumour.

Question 3

What are clinical features of Hodgkin’s lymphoma?

Answer 3

Fever, weight loss, sweating.

Question 4

What are the most common rearrangements & genes for following:?

Burkitt lymphoma (BL)
Follicularvlymphoma (FL)
DLBCL
B-cell Lyn, unclassifiable
Anaplastic large cell lymphoma, ALK +be

Answer 4

Burkitt - t(8;14) & variants, MYC
Follicular- t(14;18),   BCL2
DLBCL- various incl. t(3;14), BCL6
B-cell Lym, unclassifiable, double-hit, MYC & BCL2
Mantle cell lymphoma- t(11;14), CCND1
MALT - t(11;18), MALT1, API1
Anaplastic large cell lym, t(2;5), ALK

Question 5

What are the 3 forms of Burkitts?

Answer 5

• Sporadic: occurs globally
• Endemic: occurs in Africa, affects jaws & facial bones
• Immunodeficiency related: eg. In AIDS patients

Question 6

What is major genetic translocation in Burkitts & variants?

Answer 6

(1) 85% t(8;14)(q24;q32), MYC/IGH
(2) . 10% t(8;22), MYC, IGL
(3) . 5% t(2;8), MYC/IGK

Question 7

What is treatment for follicular lymphoma?

Answer 7

Rituximab: binds to CD20 & triggers an immune response causing apoptosis of CD20+ cells.

Question 8

Genetics of follicular lymphoma?

Answer 8

80% have t(14;18)(q32;q21)
IGH-BCL2
Rare variants: t(2;18) & t(18;22)
- 60% patients have 6q21 del
Del(17)(p13p13) seen in 20%, incl. TP53 Gene
- del(6q) & 17p abnorm, May correlate with disease transformation & poor prognosis.

Question 9

What is major translocation & secondary in Mantle Cell lymphoma?

Answer 9

• t(11;14), IGH-CCND1
• del(13q) in 40/50%
• Del of TP53 (20-45%)

Question 10

What are the 3 clinicopathological forms of Marginal zone lymphoma?

Answer 10

(1) . MALT (mucosa assoc. lymphoid tissue lymphoma): most often involve stomach, following Helicobacter pylori infection. MLT is most common MZL, 7%.
(2) Splenic MZL: presents with splenomegaly
(3) . Nodal MZL: presents advanced stage disease.