Table 3.

Use of anti-infectious regimens in patients with MZLs

PathogenMZL subtype, organPrevalence range (%)Anti-infectious regimenType of evidenceORR (CR)PFSNotes
Helicobacter pylori EMZL, stomach >90% PPI, clarithromycin-based triple therapy with amoxicillin or metronidazolea >30 retrospective or prospective studies; data from >1400 pts 75% 28 mo Responses observed also in HP-negative cases (false-negative tests or other Helicobacter species) 
Chlamydophila psittaci EMZL, ocular adnexa 0%-80% Doxycyclineb or clarithromycinc >10 retrospective and 3 prospective studies; data from >100 pts 45%-65% 55% at 5 y Wide prevalence variability depending on geographical region 
Borrelia burgdorferi EMZL, skin 0%-40% Ceftriaxoned Case reports 40% NA Prevalence high in endemic areas; median 7.3% 
Campylobacter jejuni EMZL, small bowel (IPSID) up to 60% Tetracycline, metronidazole, or ampicillin Case reports NA NA Association with low socioeconomic and sanitation status 
Achromobacter xylosoxidans EMZL, lung 2%-46% NA NA NA NA Low virulence, highly resistant to antibiotics 
Hepatitis C virus EMZL, various nongastric sites; SMZL; NMZL 5%-20% DAAse Retrospective studies, 1 prospective study 48% (26%) 73% at 3 y ORR higher in EMZL 
PathogenMZL subtype, organPrevalence range (%)Anti-infectious regimenType of evidenceORR (CR)PFSNotes
Helicobacter pylori EMZL, stomach >90% PPI, clarithromycin-based triple therapy with amoxicillin or metronidazolea >30 retrospective or prospective studies; data from >1400 pts 75% 28 mo Responses observed also in HP-negative cases (false-negative tests or other Helicobacter species) 
Chlamydophila psittaci EMZL, ocular adnexa 0%-80% Doxycyclineb or clarithromycinc >10 retrospective and 3 prospective studies; data from >100 pts 45%-65% 55% at 5 y Wide prevalence variability depending on geographical region 
Borrelia burgdorferi EMZL, skin 0%-40% Ceftriaxoned Case reports 40% NA Prevalence high in endemic areas; median 7.3% 
Campylobacter jejuni EMZL, small bowel (IPSID) up to 60% Tetracycline, metronidazole, or ampicillin Case reports NA NA Association with low socioeconomic and sanitation status 
Achromobacter xylosoxidans EMZL, lung 2%-46% NA NA NA NA Low virulence, highly resistant to antibiotics 
Hepatitis C virus EMZL, various nongastric sites; SMZL; NMZL 5%-20% DAAse Retrospective studies, 1 prospective study 48% (26%) 73% at 3 y ORR higher in EMZL 
a

PPI (standard dose) twice daily + clarithromycin 500 mg twice daily + amoxicillin 1000 mg twice daily or metronidazole 500 mg twice daily, for 14 days.

b

Doxycycline 100 mg twice daily for 3 weeks.

c

Clarithromycin 500 mg twice daily for 6 months.

d

Ceftriaxone intravenously 2 g/d for 2 weeks.

e

Sofosbuvir/velpatasvir 400/100 mg/d for 12 weeks or glecaprevir/pibrentasvir 300/100 mg/d for 8 weeks (12 weeks for genotype 3).

CR, complete response; IPSID, immunoproliferative small intestine disease; NA, not available; PPI, proton pump inhibitor; pts, patients.

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