Nonsecretory variant of immunoproliferative small intestinal disease: a case report with pathologic, immunophenotypic, and molecular findings.
We report a case of the nonsecretory variant of immunoproliferative small intestinal disease involving the distal small bowel and the mesenteric and retroperitoneal lymph nodes in a 19-year-old woman from Mexico. This variant extranodal marginal zone B-cell lymphoma appeared similar in the different sites of involvement, with more interspersed large cells and greater plasmacytic differentiation present in intestinal specimens. Characteristic lymphoepithelial lesions and follicular colonization were seen in intestinal and lymph node sections, respectively. The neoplastic B cells were cytoplasmic immunoglobulin (Ig) A heavy-chain restricted and lacked surface and cytoplasmic light-chain expression by flow cytometric analysis. Serum and urine protein electrophoresis/immunofixation revealed hypogammaglobulinemia with no paraprotein. Molecular studies showed absence of immunoglobulin heavy-chain (IgH) gene rearrangement, with a nonfunctional clonotypic rearrangement of the kappa light-chain gene. This case highlights the role for kappa light-chain gene evaluation in immunoproliferative small intestinal disease, because IgH gene rearrangement analysis is often negative.
Pai
Reetesh K
RK
Department of Pathology, University of New Mexico Health Sciences Center, Albuquerque, NM 87131, USA.
Snider
Warren K
WK
Starkey
Cindi R
CR
Viswanatha
David
D
Foucar
M Kathryn
MK
Wilson
Carla S
CS
D053799:2-Pyridinylmethylsulfinylbenzimidazoles; D000328:Adult; D000658:Amoxicillin / Q000627:therapeutic use; D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D001562:Benzimidazoles / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D005260:Female; D015328:Gene Rearrangement, B-Lymphocyte, Light Chain / Q000235:genetics; D006801:Humans; D016130:Immunophenotyping; D007161:Immunoproliferative Small Intestinal Disease / Q000235:genetics / Q000276:immunology / Q000473:pathology*; D007421:Intestine, Small / Q000473:pathology; D008198:Lymph Nodes / Q000473:pathology*; D018442:Lymphoma, B-Cell, Marginal Zone / Q000473:pathology*; D008643:Mesentery; D008795:Metronidazole / Q000627:therapeutic use; D009853:Omeprazole / Q000031:analogs & derivatives / Q000627:therapeutic use; D000077402:Pantoprazole; D012187:Retroperitoneal Space; D013454:Sulfoxides / Q000627:therapeutic use
D053799:2-Pyridinylmethylsulfinylbenzimidazoles; D000900:Anti-Bacterial Agents; D001562:Benzimidazoles; D013454:Sulfoxides; D008795:Metronidazole; D000658:Amoxicillin; D000077402:Pantoprazole; D009853:Omeprazole