Extranodal Marginal Zone B Cell Lymphoma
Variant: Immunoproliferative Small Intestinal Disease
Definition of IPSID
- Subtype of extranodal marginal zone lymphoma involving the small intestine, with alpha heavy chain production
Alternate/Historical Names for IPSID
- IPSID
- Alpha heavy chain disease
- Mediterranean lymphoma
Diagnostic Criteria for IPSID
- Lymphoplasmacytic infiltrate
- Usually prominently plasmacytic
- Lymphoepithelial lesions and follicular colonization may be prominent
- Involves mucosa, may extend into submucosa
- Mesenteric lymph node involvement common
- May transform to large cell lymphoma
- May retain plasmacytic differentiation
- Typically forms tumor masses
Immunologic Stains for IPSID
- Alpha heavy chain positive
- No light chain
- Otherwise similar to usual marginal zone lymphoma
Clinical Findings of IPSID
- Predominantly Mediterranean in distribution
- Also reported in South Africa
- Sporadic reports in other sites
- Predominantly young adults
- Presents with diarrhea (steatorrhea) and weight loss
- Alpha heavy chain may be detected in serum
- An association with Campylobacter jejuni infection has been reported
