Roles of syndecan-1, bcl6 and p53 in diagnosis and prognostication of immunoproliferative small intestinal disease.
OBJECTIVE
To evaluate roles of syndecan-1, bcl6 and p53 in diagnosis and prognostication of immunoproliferative small intestinal disease (IPSID) and to study profiles of kappa (kappa) and lambda (lambda) light chains and IgA heavy chain.


METHODS
The study consisted of 11 cases of IPSID and similar number of controls which included 11 of normal intestinal mucosa and 11 of high grade B cell lymphoma of ileum. The parameters analyzed included clinical profiles, biochemical and other laboratory investigations, radiologic and histological findings including immunohistochemistry.


RESULTS
All IPSID cases had demonstrable serum IgA heavy chain and heavy mucosal plasma cell infiltration. According to Galian's histological staging, there were 4 patients with stage A and 7 with stage B. kappa and lambda light chains were over-expressed in 7 patients; 1 stage A patient had H pylori-positive active gastritis and eradication of H pylori led to disease remission. Stage A biopsies had higher expression for syndecan-1, while stage B had higher expression for bcl6 and p53. Syndecan-1, kappa and lambda light chains and IgA heavy chain showed inverse relationship with bcl6 and p53. All patients were treated with doxycycline. CHOP regime was added in 5 patients who developed frank lymphoma. Three died of the disease due to extensive organ infiltration.


CONCLUSIONS
Certain immunomarkers like syndecan-1, kappa and lambda light chains and IgA heavy chain could be of much help in identifying early stage IPSID. Stage B IPSID showed higher expression for bcl6 and p53 than stage A IPSID. bcl6 and p53 expressions correlated with a more advanced disease stage and aggressive tumour behavior.
Vaiphei
Kim
K
Department of Histopathology, PGIMR, Chandigarh, India. kvaiphei@gmail.com
Kumari
Neeraj
N
Sinha
Saroj-Kant
SK
Dutta
Usha
U
Nagi
Birinder
B
Joshi
Kusum
K
Singh
Kartar
K
D000328:Adult; D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D016022:Case-Control Studies; D004268:DNA-Binding Proteins / Q000032:analysis / Q000235:genetics* / Q000276:immunology; D018450:Disease Progression; D004318:Doxycycline / Q000627:therapeutic use; D016099:Endoscopy, Gastrointestinal; D005260:Female; D005786:Gene Expression Regulation; D016480:Helicobacter pylori; D006801:Humans; D007137:Immunoglobulin alpha-Chains / Q000097:blood / Q000235:genetics; D007145:Immunoglobulin kappa-Chains / Q000032:analysis / Q000235:genetics; D007146:Immunoglobulin lambda-Chains / Q000032:analysis / Q000235:genetics; D007150:Immunohistochemistry; D007161:Immunoproliferative Small Intestinal Disease / Q000175:diagnosis* / Q000188:drug therapy / Q000235:genetics* / Q000276:immunology; D007413:Intestinal Mucosa / Q000737:chemistry / Q000382:microbiology / Q000473:pathology; D007421:Intestine, Small / Q000737:chemistry / Q000382:microbiology / Q000473:pathology; D008297:Male; D008562:Membrane Glycoproteins / Q000032:analysis / Q000235:genetics* / Q000276:immunology; D008875:Middle Aged; D011379:Prognosis; D011509:Proteoglycans / Q000032:analysis / Q000235:genetics* / Q000276:immunology; D051560:Proto-Oncogene Proteins c-bcl-6; D053668:Syndecan-1; D053667:Syndecans; D016159:Tumor Suppressor Protein p53 / Q000032:analysis / Q000235:genetics* / Q000276:immunology
D000900:Anti-Bacterial Agents; C084103:BCL6 protein, human; D004268:DNA-Binding Proteins; D007137:Immunoglobulin alpha-Chains; D007145:Immunoglobulin kappa-Chains; D007146:Immunoglobulin lambda-Chains; D008562:Membrane Glycoproteins; D011509:Proteoglycans; D051560:Proto-Oncogene Proteins c-bcl-6; C508241:SDC1 protein, human; D053668:Syndecan-1; D053667:Syndecans; D016159:Tumor Suppressor Protein p53; D004318:Doxycycline