Indeterminate Dendritic Cell Tumor (IDCT) of GI Tract, Lymph Node and Spleen Masquerading as Refractory Inflammatory Bowel Disease

2020 ◽  
Vol 154 (Supplement_1) ◽  
pp. S108-S108
Author(s):  
W Huang ◽  
M G Bayerl
Keyword(s):  
Inflammatory Bowel Disease ◽  
Lymph Node ◽  
Dendritic Cell ◽  
Tumor Cells ◽  
Bowel Disease ◽  
Langerhans Cells ◽  
Cell Tumor ◽  
Gi Tract ◽  
Medical Treatments ◽  
Inflammatory Bowel

Abstract Introduction/Objective Indeterminate dendritic cell tumor (IDCT) is an extremely rare neoplasm, most frequently presenting in skin with cells resembling precursors to Langerhans cells. We report an exceptional case of IDCT occurring in GI tract, spleen, and lymph nodes of a 66-year-old lady, mimicking inflammatory bowel disease (IBD). Methods Four years prior to the diagnosis of IDCT, she was diagnosed with ulcerative colitis (UC) based on bloody diarrhea and pan-colitis. Her colitis became refractory to medical treatments and she developed pancytopenia, splenomegaly and abdominal lymphadenopathy. A FNA of abdominal lymph node and a colonoscopic biopsy both showed non-necrotizing granulomas, which along with her multiple oral ulcers suggested Crohn’s disease. Due to failure to medical treatments, she underwent proctocolectomy and ileostomy, followed later by subtotal colectomy with excision of a splenule and lymph nodes. Results All specimens from the final operation showed involvement by IDCT characterized by polygonal cells with abundant eosinophilic cytoplasm, oval nuclei with occasional nuclear grooves, open chromatin and eosinophilic nucleoli. By immunohistochemistry, the tumor cells expressed S100, CD1a, cyclin D1, BRAFV600E. Langerin staining was observed in <5% of cells, suggesting partial differentiation of tumor cells (frequently seen in IDCT) vs. reactive Langerhans cells. No overt cytological atypia and no necrosis were observed. Eosinophils, neutrophils, lymphocytes and plasma cells were sparse. There was no emperioloperesis. The colon showed multifocal active and chronic inflammation and ulcerations associated with the IDCT infiltrate. These specimens were diagnostic of IDCT. Conclusion Absence Langerin in the majority of cells excluded the possibility of Langerhans cell histiocytosis. The presence of CD1a and absence of bone involvement excluded the possibility of Erdheim-Chester disease. Retrospectively, it appears that the patient’s symptoms were due to IDCT rather than IBD. The clinical course of IDCT is highly variable, but the BRAF mutation offers a promising new therapeutic target for this patient.

Inflammatory bowel disease

2018 ◽  
Author(s):  
Satish Keshav ◽  
Alexandra Kent
Keyword(s):  
Inflammatory Bowel Disease ◽  
Bowel Disease ◽  
Age Of Onset ◽  
Anal Verge ◽  
Age At Onset ◽  
Mucosal Inflammation ◽  
Gi Tract ◽  
Disease Extent ◽  
Inflammatory Bowel ◽  
Different Characteristics

Inflammatory bowel disease (IBD) encompasses ulcerative colitis (UC) and Crohn’s disease (CD). Both conditions cause chronic relapsing inflammation in the gastrointestinal (GI) tract, but have different characteristics. UC causes diffuse mucosal inflammation limited to the colon, extending proximally from the anal verge, with the rectum involved in 95% of patients. UC is described in terms of the disease extent: proctitis (confined to the rectum), proctosigmoiditis (disease confined to the recto-sigmoid colon), distal disease (distal to the splenic flexure), and pan-colitis (the entire large intestine). The extent of disease can change, with proximal extension seen in approximately a third of patients with proctitis, although there is great variation between studies. CD causes inflammation that can affect the entire thickness of the wall of the intestine, and is not confined to the mucosa. CD can affect any part of the GI tract. The terminal ileum is affected in approximately 80% of cases, the colon in approximately 60% of cases, and the rectum and perianal region in approximately 40% of cases. CD is classified by location (ileal, colonic, ileocolonic, upper GI tract), by the presence of stricturing or penetrating disease, and by the age of onset (before or after the age of 40). Penetrating disease refers to the development of fistulae, which can lead to complications such as abscesses or perforations. An earlier age at onset is associated with more complicated disease. The diagnosis of UC or CD is established through a combination of clinical, endoscopic, radiological, and histological criteria rather than by any single modality. Occasionally, it is not possible to establish an unequivocal diagnosis of CD or UC in IBD, and a third category, accounting for nearly 10% of cases, is used, termed IBD unclassified.

Effects of Tumor Necrosis Factor Alpha Inhibitors on Lymph Node and Ileocecal Mucosa-Associated Lymphoid Tissue Architecture in Patients With Inflammatory Bowel Disease

2019 ◽  
Vol 23 (2) ◽  
pp. 115-120
Author(s):  
Virginia E Duncan ◽  
Karen M Chisholm ◽  
M Cristina Pacheco
Keyword(s):  
Inflammatory Bowel Disease ◽  
Lymph Node ◽  
Germinal Center ◽  
Bowel Disease ◽  
Lymphoid Tissue ◽  
Ileocecal Valve ◽  
Tissue Architecture ◽  
Mesenteric Lymph ◽  
Tnfα Inhibitors ◽  
Inflammatory Bowel

Background Antitumor necrosis alpha (TNFα) therapy is often used in the management of patients with inflammatory bowel disease (IBD) and may have effects on lymphoid tissue architecture and function. The goal of our study was to characterize the effects of TNFα inhibitors on mesenteric lymph node and mucosa-associated lymphoid tissue in patients with IBD. Methods We examined lymphoid tissue morphology in IBD patients treated with TNFα inhibitors compared to untreated controls. Intestinal resections from 19 patients (10 anti-TNFα treated and 9 controls) were reviewed. Immunohistochemistry for CD21, CD20, and CD3 was performed on ileocecal valve lymphoid tissue and mesenteric lymph nodes from the resection specimens to assess follicular architecture. Results Relative to control groups, TNFα-treated groups showed less preserved germinal center architecture, evidenced by lower overall semiquantitative scores for follicular architecture. Likewise, the percentage of secondary follicles to total follicles was decreased in patients treated with TNFα blockade. Conclusions Our results suggest that TNFα inhibitors may play a role in disruption of lymphoid germinal center architecture in patients with IBD. Awareness of this disrupted lymphoid morphology when examining histologic sections from patients with IBD treated with TNFα inhibitors may prevent unnecessary studies to exclude a lymphoproliferative disorder.

scholarly journals Dendritic cell profiles in the inflamed colonic mucosa predict the responses to tumor necrosis factor alpha inhibitors in inflammatory bowel disease

2018 ◽  
Vol 52 (4) ◽  
pp. 443-452
Author(s):  
Natasa Smrekar ◽  
David Drobne ◽  
Lojze M. Smid ◽  
Ivan Ferkolj ◽  
Borut Stabuc ◽  
...  
Keyword(s):  
Inflammatory Bowel Disease ◽  
Dendritic Cells ◽  
Dendritic Cell ◽  
Tumor Necrosis Factor ◽  
Bowel Disease ◽  
Tumor Necrosis ◽  
Necrosis Factor Alpha ◽  
Inflammatory Bowel ◽  
Factor Alpha ◽  
Necrosis Factor

Abstract Background Dendritic cells play crucial roles in the control of inflammation and immune tolerance in the gut. We aimed to investigate the effects of tumor necrosis factor alpha (TNFa) inhibitors on intestinal dendritic cells in patients with inflammatory bowel disease and the potential role of intestinal dendritic cells in predicting the response to treatment. Patients and methods Intestinal biopsies were obtained from 30 patients with inflammatory bowel disease before and after treatment with TNFa inhibitors. The proportions of lamina propria dendritic cell phenotypes were analysed using flow cytometry. Disease activity was endoscopically assessed at baseline and after the induction treatment. Results At baseline, the proportion of conventional dendritic cells was higher in the inflamed mucosa (7.8%) compared to the uninflamed mucosa (4.5%) (p = 0.003), and the proportion of CD103+ dendritic cells was lower in the inflamed mucosa (47.1%) versus the uninflamed mucosa (57.3%) (p = 0.03). After 12 weeks of treatment, the proportion of conventional dendritic cells in the inflamed mucosa decreased from 7.8% to 4.5% (p = 0.014), whereas the proportion of CD103+ dendritic cells remained unchanged. Eighteen out of 30 (60%) patients responded to their treatment by week 12. Responders had a significantly higher proportion of conventional dendritic cells (9.16% vs 4.4%, p < 0.01) with higher expression of HLA-DR (median fluorescent intensity [MFI] 12152 vs 8837, p = 0.038) in the inflamed mucosa before treatment compared to nonresponders. Conclusions A proportion of conventional dendritic cells above 7% in the inflamed inflammatory bowel disease mucosa before treatment predicts an endoscopic response to TNFa inhibitors.

scholarly journals Bacteria, good and bad: Host–microbiota interactions in inflammatory bowel disease

2011 ◽  
Vol 33 (4) ◽  
pp. 22-25
Author(s):  
Paul Flanagan ◽  
Barry J. Campbell ◽  
Jonathan M. Rhodes
Keyword(s):  
Inflammatory Bowel Disease ◽  
Crohn’S Disease ◽  
Crohn's Disease ◽  
Bowel Disease ◽  
Major Surgery ◽  
Medical Treatments ◽  
Transmural Inflammation ◽  
Inflammatory Bowel ◽  
The Uk ◽  
Inflammatory Molecules

Inflammatory bowel disease (IBD), a term encompassing the conditions ulcerative colitis (UC) and Crohn's disease, affects around 1 in 300 people in the UK and causes significant morbidity, although, thankfully, little mortality1. Although UC is limited to the colon and causes mucosal ulceration, Crohn's disease can affect any part of the intestine and causes ulceration, transmural inflammation, stricturing, fistula and abscess formation. Both are relapsing and remitting diseases and, despite advances in medical treatments, including immunosuppressants and drugs which specifically block pro-inflammatory molecules (tissue necrosis factor; TNF), about 25% with UC and 50–80% with Crohn's disease will require major surgery at least once in their lives.

scholarly journals Mechanisms underlying the development of inflammatory bowel disease: the role of rankl in dendritic cell activity

Gut ◽  
2011 ◽  
Vol 60 (Suppl 1) ◽  
pp. A138-A138
Author(s):  
T. S. Chew ◽  
L. Logunova ◽  
J. McLaughlin ◽  
S. Cruickshank
Keyword(s):  
Inflammatory Bowel Disease ◽  
Dendritic Cell ◽  
Bowel Disease ◽  
Cell Activity ◽  
Inflammatory Bowel

Common Etiologies and Clinical Significance of Gastric Intraepithelial Lymphocytosis

2019 ◽  
Vol 152 (Supplement_1) ◽  
pp. S61-S62
Author(s):  
Sunjida Ahmed ◽  
Ruliang Xu
Keyword(s):  
Inflammatory Bowel Disease ◽  
Morbid Obesity ◽  
Celiac Disease ◽  
Bowel Disease ◽  
Intraepithelial Lymphocytes ◽  
Gi Tract ◽  
Inflammatory Bowel ◽  
H Pylori ◽  
Intraepithelial Lymphocytosis

Abstract Background Increased intraepithelial lymphocytes or intraepithelial lymphocytosis (IEL) in the upper gastrointestinal (GI) tract is a response to various mucosal injury. However, the frequency of gastric IEL in GI tract biopsy is not well documented, and the etiologies of gastric IEL are yet to be defined. Methods Cases with a diagnosis of “intraepithelial lymphocytosis” and “intraepithelial lymphocytes” were retrieved from 25,074 GI biopsies and 8,921 partial gastrectomies in our departmental database (Powerpath) for a 1-year period. The diagnosis of IEL was confirmed by the report and/or slide review. Possible etiology or causes of gastric IEL were investigated by correlation with clinical information from LIS (EPIC). Results A total of 694 cases with IEL were identified from 33,995 GI tract specimens (biopsy and resection). Among 694 cases, 34 (4.89%) were gastric biopsy and resection cases with IEL, whereas 561 (80.8%), 37 (5.3%), and 62 (8.9%) were duodenal, esophageal, and colonic specimens, respectively. Thirty-four gastric cases with IEL were closely associated with morbid obesity (8, 23.5%), H pylori infection (8, 23.5%), celiac disease (5, 14.7%), lymphocytic gastritis (5, 14.7%), nonspecific gastritis (4, 11.4%), inflammatory bowel disease (3, 8.8%), and gastroesophageal reflux disease (1, 1.9%). Seventeen of 34 (50%) cases had IEL in both gastric and duodenal mucosa. Those 17 cases with gastroduodenal IEL had morbid obesity (n = 5), celiac disease (n = 5), lymphocytic colitis (n = 2), inflammatory bowel disease (n = 2), H pylori gastritis (n = 2), and nonspecific gastritis (n = 1). Five patients with a diagnosis of lymphocytic gastritis were treated with a protein pump inhibitor after pathologic diagnosis. Among them, 4 had a followed-up endoscopy in 12 months, and 3 of them showed persistent IEL in a follow-up biopsy. Conclusion Gastric IEL is less common than duodenal IEL. It is associated with a broad differential diagnosis. Follow-up biopsy may be necessary for some types of gastric IEL. Persistent IEL in follow-up biopsy may be suggestive of a different etiology or requires different treatment strategy.

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