It has been postulated that chronic inflammation leads to activation of NF-κB pathway via the antigen receptor signaling in MALT lymphoma cells. Antigen stimulation and CD40 triggering synergize NF-κB activation through formation of CARMA1-BCL10-MALT1 ternary complex. In addition, the continuous and sustained antiapoptotic stimuli driven by Inhibitors,research,lifescience,medical API2-MALT1 are most likely to play key roles in the pathogenesis of MALT lymphomas (32,33). Prognosis The response of low grade

MALT lymphoma to H. pylori eradication is predicted by stage. Complete regression of low-grade, early stage MALT lymphoma following successful H. pylori eradication has been confirmed in about 75-80% of cases (4-6,35). Studies have documented that complete response has been achieved Inhibitors,research,lifescience,medical in nearly all patients

where ABT869 disease is limited to the gastric mucosa or submucosa. Complete response rates have decreased in cases where disease extended to the muscularis propria or serosa (35). Furthermore, it has been shown that no patients with nodal disease achieved complete response with H. pylori eradication alone (4-6,36-39). It is important to note, however, that approximately 10% of gastric MALT lymphomas Inhibitors,research,lifescience,medical with t[11;18] [q21;q21] translocation are resistant to H. pylori antibiotic therapy, suggesting importance of strict follow up, and if clinically indicated, a trial of chemotherapy, immunotherapy (i.e., Rituximab), and/or radiotherapy for localized disease, may be pursued (6,36-40). Studies suggest that medical therapy alone is superior to surgery, although surgical intervention may be appropriate in specific circumstances such as Inhibitors,research,lifescience,medical in cases with gastric outlet obstruction and/or other complications (35). Immunoproliferative small intestinal disease Inhibitors,research,lifescience,medical (IPSID) IPSID has also been acknowledged as alpha heavy chain disease (αHCD), and is a variant form of

MALT lymphoma arising in the small intestine. IPSID or αHCD is the most common form of the heavy chain diseases (HCD). It accounts for about one-third of all GI lymphomas in the middle-east. IPSID occurs in a younger age population, with most patients presenting at the age of 20 to 30 years (7). Pathogenesis As in cases of H. pylori associated MALT lymphoma, an infectious etiology has been suspected in cases of Cytoskeletal Signaling inhibitor IPSID. Studies have mirrored the efficacy of antimicrobial therapy in disease regression. Lecuit et al. demonstrated C. jejuni as a possible stimulus for this proliferation. C. jejuni has been shown to persist in Peyer’s patches and mesenteric lymph nodes, and is capable of eliciting strong IgA mucosal response. Persistent infection may lead to sustained stimulation of B cells eventually resulting in the production of monotypic IgA such as that seen in IPSID (7).