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Flashcards in Marginal Zone Lymphomas Deck (6):
1

What are the 3 distinct subtypes of MZLs?

1) Extranodal MZL of mucosa-associated lymphoid tissue (MALT)
2) Nodal MZL
3) Splenic MZL

2

What are some common infectious pathogens a/w MZLs?

H.Pylori
Chlamydia psittaci
Campylobacter jejuni
Borrelia Burgdorferi
Hep C

3

How often is Hep C a/w MZLs?

30%
Esp splenic MZL

HCV has also be reported in about 35% of patients with non-gastric MALTs

4

What is commonly difficult to separate from MZL as a diagnosis?

Any mutations you know of that can help?

Waldenstrom's Macroglobulinemia/Lymphoplasmacytic lymphoma (WM/LPL)

MYD88 L265P somatic mutation is widely prevalent in WM/LPL patients


IGHV analysis clearly distinguishes splenic MZLs and WM/LPL
- Splenic MZLs were characterized by over representation of IGHV1-2 gene rearrangements with low/intermediate mutation rates
- WM/LPL a/w over-representation of IGHV3-23 rearrangements and high mutation rates.

5

Tell me about the epidemiology of MALT

GI tract most common site of involvement. 50% of MALT lymphomas.

Within GI tract, stomach 80-85%

Other common non-gastric sites include: Orbit, lung, skin

MALT lymphomas tend to be indolent.
Similar long-term outcomes between gastric and non-gastric subtypes.

T(11;18) most common translocation, resulting in formation of chimeric fusion Gene, API2-MALT1
- frequently detected in gastric, pulmonary MALT lymphomas

T(1;14) results in overexpression of BCL10 protein and it occurs in 1-2% of MALT lymphomas.

T(11;18) and BCL10 overexpression a/w locally advanced disease and is less likely to respond to H.Pylori eradication with antibiotic therapy.

T(14;18) results in deregulated expression of MALT1 Gene
- in 15-20% of MALT lymphomas

T(3;14) results in up emulation of FOXP1 Gene, a/w MALT of thyroid, ocular adnexa and sin

6

What is the Lugano staging system for GI lymphomas?

Stage IE = confined to GI tract (can be single or multiple, non contiguous)

Stage IIE = extending into abdomen

Stage IIE + T4N0M0= penetration of serous a to involve adjacent organs/tissues

Stage IV = disseminated Extranodal involvement or concomitant supra diaphragmatic nodal involvement