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Casus 2

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.

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Casus 2

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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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