Question
Which of the following is the most prominent feature of Immunoproliferative Small Intestinal Disease (IPSID)?
| A. |
Malabsorption |
| B. |
Obstruction |
| C. |
Bleeding |
| D. |
Abdominal pain |
Show Answer
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Correct Answer � A Explanation |
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Ans.A. Malabsorption
- Immunoproliferative small intestinal disease (IPSID)was recently added to the growing list of infectious pathogen-associated human lymphomas.
- Molecular and immunohistochemical studies demonstrated an association with Campylobacter jejuni.
- IPSID is a variant of the B-cell lymphoma of mucosa-associated lymphoid tissue (MALT), which involves mainly the proximal small intestine resulting in malabsorption, diarrhea, and abdominal pain.
- Geographically, IPSID is most prevalent in the Middle East and Africa.
- IPSID lymphomas reveal excessive plasma cell differentiation and produce truncated α heavy chain proteins lacking the light chains as well as the first constant domain.
- The corresponding mRNA lacks the variable heavy chain (VH) and the constant heavy chain 1 (CH1) sequences and contains deletions as well as insertions of unknown origin.
- The encoding gene sequence reveals deletion of the V region and parts of the CH1 domain.
- Cytogenetic studies demonstrated clonal rearrangements involving predominantly the heavy and light chain genes, including t(9;14) translocation involving the PAX5 gene.
- Early-stage IPSID responds to antibiotics (30%-70% complete remission).
- Most untreated IPSID patients progress to lymphoplasmacytic and immunoblastic lymphoma invading the intestinal wall and mesenteric lymph nodes and may metastasize to a distant organ.
- IPSID lymphoma shares clinical, morphologic, and molecular features with MALT lymphoma, lymphoplasmacytic lymphoma, and plasma cell neoplasms.
