Treatment-Resistant Immunoproliferative Small Intestinal Disease (IPSID) Leading to Lymphoma

Area of Science:

• Gastroenterology
• Oncology
• Infectious Diseases

Background:

• Immunoproliferative small intestinal disease (IPSID) is a variant of mucosa-associated lymphoid tissue (MALT) lymphoma, often associated with chronic Campylobacter jejuni infection.
• IPSID is an extra-nodal marginal zone B-cell lymphoma primarily affecting the proximal small intestine, characterized by lymphoplasmacytic infiltration and monotypic alpha-heavy chains.

Observation:

• A case study of a Nigerian woman with IPSID presented with refractory watery diarrhea, abdominal pain, and weight loss.
• Diagnostic findings included positive Campylobacter stool antigen, mesenteric lymphadenopathy, and small bowel biopsy showing lymphoplasmacytic infiltration with villi flattening.

Findings:

• The patient initially responded to antibiotics, but experienced frequent recurrences, hospitalizations, and ultimately disease progression to lymphoma.
• Distinguishing IPSID from other causes of mesenteric lymphadenopathy proved complex, highlighting diagnostic challenges.

Implications:

• This case underscores the need for heightened clinical awareness of IPSID in endemic regions and the importance of rigorous monitoring to prevent malignant transformation.
• Further research is needed to understand IPSID progression mechanisms and improve therapeutic strategies to prevent progression to lymphoma despite antibiotic treatment.