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Understanding Gastric MALT Lymphoma: Diagnosis, Treatment & Prognosis

Learn about Gastric MALT Lymphoma diagnosis, importance of H. pylori, treatment options, and positive prognosis.

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Understanding Gastric MALT Lymphoma: Diagnosis, Treatment & Prognosis

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  1. Casus 2 • Een 40 jarige man bezoekt de huisarts i.v.m. sinds een half jaar bestaande klachten van zuurbranden en pijn midden in de bovenbuik, vooral na de maaltijd. • De huisarts schrijft een protonpomp remmer voor. Dit resulteert wel in enige verbetering, maar de klachten blijven bestaan. • Patiënt wordt verwezen voor een gastroscopie. In het antrum wordt een ulcererende zwelling gezien van 1,5x 2 cm. Er worden multipele biopten genomen

  2. Immunohistochemische kleuring met anti CD20 antistoffen. HE kleuring

  3. Casus 2 Vragen: • Welke diagnose overweegt U en wat zijn hiervoor belangrijke argumenten? • Welk micro-organisme speelt een belangrijke rol in de pathogenese? • Wat is de behandeling?

  4. Gastric MALT lymphomakey messages • Distinct disease entity • Pivotal role of chronic antigenic stimulation by H. pylori • Can be cured by antibiotic treatment • Good prognosis (5 yrs OS 82-93%)

  5. MALT: Mucosa-Associated Lymphoid Tissue Can be induced/expanded by chronic antigenic stimulation Lymphomas of MALT-type : ~8% of all NHL Two subgroups Gastric MALT Lymphomas (70%) Non-Gastric MALT Lymphomas (30%) MALT Lymphoma

  6. Gastric MALT Lymphoma:History • 1991 Wotherspoon et al. • Association H.Pylori gastritis and Gastric MALT lymphoma • 90% H.Pylori infection, • 98% H.Pylori positive serology • 1993 Wotherspoon et al. • Remission of MALT lymphoma after H.pylori eradication • 1996 Hussell et al. • H.Pylori strain specific T-cells involved in lymphomagenesis

  7. Gastric MALT Lymphoma:Gastroscopy

  8. Gastric MALT Lymphoma:Histology • LEL’s (Lymphoepithelial lesions) • monoclonal small/meduim sized B cells (“marginal zone cells”) • CD20+,CD79a+,CD5-,CD10-, CD23-, CD21+, CD35+,IgM+ • Plasmacytoid differentiation

  9. Translocations in MALT lymphoma 13.5% 10.8% 1.6% All result in antigen-independent NFkB activation - proliferation - Inhibition of apoptosis t(11;18) t(14;18) t(1;14) Unknown! 74.1% n=252 Streubel et al., Leukemia 2004

  10. Pathogenesis Gastric MALT Lymphoma: HP Infectionacquired MALT HP-specific T-cell APC HP dependent NFkB activation of B cells - proliferation - Inhibition of apoptosis B-cell MALT lymphoma Chromosomal translocations → HP independent NFkB activation -

  11. Gastric MALT Lymphoma: assessment of localisations • Gastroscopy with multiple biopsies (H.Pylori culture) • Endosonography of the stomach • CT-chest and abdomen (gastric protocol) • Ophthalmologic and ENT-examination • Bone Marrow investigation • Further Investigation of GI-tract depending on symptoms 25 % also extragastric localisation !

  12. Gastric MALT LymphomaTherapy local disease • H.Pylori eradication with strict Follow-Up • Omeprazole 20 mg bid d1-7, • Amoxycillin 1000 mg bid d1-7,  CR 70-80% • Clarithromycin 500 mg bid d1-7 • Similar OS with different treatments : 5yrs OS 82% • chemotherapy,surgery, surgery with additional chemotherapy or radiationtherapy or H.Pylori eradication

  13. Effect of eradication of H.Pylori Before Hp eradication 2 weeks post-eradication 10 months post-eradication Dr Naomi Uemura, Hiroshima Japan

  14. Gastric MALT LymphomaTherapy II • Radiotherapy: • Chemotherapy (mild, oral) • Immunotherapy: Rituximab Advanced disease: • Comparable with follicular lymphoma: • CVP-R or FCR

  15. Non Gastric MALT lymphomas:

  16. Pathogenesis non-gastric MALT ymphoma Bacterial Infection Auto-antigen APC T-cell B-cell MALT lymphoma

  17. Multistage development of gastric MALT lymphoma Isaacson et al. Nature Rev. Cancer 2004:4;644-653

  18. T4 N1 regional Adjacent structures or organs N2 intra-abdominal separate GI site separate non-GI site M1 M2 N3 extra-abdominal Paris Staging system of Gastric MALT Lymphoma T1 T2 T3 mucosa m.mucosa submucosa m.propria serosa B0 BM neg B1 BM pos

  19. Predictors of response toHelicobacter Pylori eradication • Depth of invasion of gastric wall • Helicobacter status at diagnosis • Presence/absence of large cell component • Immunocytochemistry • nuclear bcl-10 • nuclear NF-kB • Molecular abnormalities • API-2/MALT-1 fusion t(11;18) • t(1;14) • Trisomy 3

  20. Non Gastric MALT Lymphoma:Therapy and Prognosis • No randomized controlled trials  • Patient tailored therapy • Local disease: • Radiotherapy • Chemotherapy, Immunotherapy, Surgery • Advanced disease: “indolent lymphomas” • Prognosis: 5 year survival 82-93%

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