Marginal Zone Lymphoma

Question 1

MZL - Work up

Answer 1

• For gastric - needs 10 biopsy for lesion as well as random biopsy.
• EUS used for nodal status
• Staging for all - CT imaging
• PET can miss disease (up to 60%)
• SPEP (esp if plasmacytoid appearance)
• Consider marrow even without cytopenias to r/o upstage

Question 2

MZL - ENMZL presentation

Answer 2

8% of NHL
~ 60 year at presentation (30-90 y/o)
- 40% stage I, 30% stage II
- Locations
   - 45% gastric
   - 7% small intestine
   - 12% occular
   - 11% bronchial mucosa
   - 9% skin
   - 6% salivary
   - 6% thyroid

Question 3

MZL - IPSID

Answer 3

Immunoproliferative small intestinal disease

• proximal small bowel diffusely effected
• middle eastern/north africa
• presents with malabsorption, fever, and pain

Question 4

MZL - histology

Answer 4

• small B cells with scant cytoplasm
• abundance of larger cells should r/o DLBCL
• lymphoid infiltration with glandular destruction (lymphoepithelioid lesions) suggest lymphoma rather than lymphoid hyperplasia

Question 5

MZL - immunophenotype

Answer 5

CD19, CD20 postiive
CD21, CD35 and CD79a positive
CD138 can be positive for plasmcytoid cells
Typically CD5 neg, CD23 neg
CD10 neg (in contrast to FL)
neg BCL6 (contrast with DLBCL)

Question 6

MZL - early stage gastric

Answer 6

• Treat h pylori and confirm eradication
• F/u 3 month later EGD to confirm response
• PR or overt residual disease if asymptomatic as response can occur up to 18 month later
• Asymptomatic tx with XRT 30 Gy
• Follow up EGD 3-6 month for 5 year
• 7% 5 year relapse if CR after H pylori eradication

Question 7

MZL - Occular tx

Answer 7

• Can try tetracycline to treat ch psittaci

- otherwise XRT

Question 8

MZL - Prognosis

Answer 8

IPI can possibly predict outcomes

• 10% can transform to DLBCL
• typically >90% 5 year OS