[Embolic stroke due to ascending aortic thrombus in a patient with treatment-resistant ulcerative colitis].

The patient was a 49-year-old man presenting with recurrent melena due to progressive ulcerative colitis. One day, he developed left lower facial weakness and dysarthria, and the next day, he was transferred to our hospital because of muscle weakness in his left upper and lower extremities. On admission, neurological findings revealed left hemiplegia, including left facial palsy, dysarthria, and left hemispatial neglect. Brain MRI with diffusion-weighted image showed a fresh infarction in the right anterior and middle cerebral artery territory. Contrast-enhanced CT showed thrombus in the ascending aorta in addition to occlusion of the right internal carotid artery, suggesting the diagnosis of cerebral infarction with an embolic source in the aortic lesion. The intra-aortic thrombus disappeared after 48th day of antithrombotic therapy. Laboratory findings revealed elevated blood viscosity, proteinase-3-anti-neutrophil cytoplasmic antibody (PR3-ANCA), and ß2GP1-IgG antibodies, suggesting that the cause of the aortic thrombus may be due to elevated blood viscosity and autoantibodies, as well as highly active ulcerative colitis.

Resolution of Laryngeal Ulceration with Prednisolone Therapy in a Patient with Crohn's Disease.

A 23-year-old man diagnosed with Crohn's disease (CD) was treated with infliximab. He developed new-onset sore throat and dysphagia during admission, and nasopharyngoscopy revealed epiglottic ulceration. Laryngeal ulceration was considered as an extraintestinal manifestation of CD owing to treatment failure with antibiotics and hydrocortisone. This strongly suggested that laryngeal ulceration was a complication of CD because of the rapid improvement in the symptoms and lesions after prednisolone administration. Furthermore, this treatment process demonstrated the superior anti-inflammatory effect of prednisolone over that of hydrocortisone and supported the assumption of inflammation related to CD.

Malnutrition and inflammation status in nonobese patients with inflammatory bowel disease are associated with nonalcoholic fatty liver disease: a retrospective study.

BACKGROUND/AIMS: The frequency and details of nonalcoholic fatty liver disease (NAFLD) complications in patients with inflammatory bowel disease (IBD) remain unclear. This study aimed to clarify characteristics of NAFLD in patients with IBD. METHODS: We retrospectively identified and enrolled patients with IBD diagnosed with or without NAFLD by undergoing abdominal computed tomography (CT) at our institution between 2005 and 2020. The primary endpoint was the complication rate of NAFLD in patients with IBD. Secondary endpoints were the clinical characteristics of nonobese patients with IBD and comorbid NAFLD and their association with nutritional and inflammatory parameters. RESULTS: Twenty-one (21.9%) of 96 eligible patients with IBD also had NAFLD. In nonobese patients (defined as patients with a body mass index <25 kg/m2), C-reactive protein (CRP; P<0.001) and alanine aminotransferase (P=0.018) levels were higher and the albumin level (P=0.005) and prognostic nutritional index (PNI; P=0.002) values were lower in patients with NAFLD than in those without NAFLD. The PNI value was positively correlated (P<0.001) and the CRP level was negatively correlated (P=0.001) with the hepatosplenic ratio. However, in the NAFLD combined group, PNI (P<0.05) and CRP values (P<0.001) were improved over time after CT imaging by continuing IBD treatment. CONCLUSIONS: Worsening nutritional and inflammatory status in IBD patients is associated with complications of NAFLD. Diagnosis of NAFLD in IBD patients using CT imaging might be useful not only for early detection of NAFLD but also in assessing the need for therapeutic intervention for IBD.

[Clinicopathological features of serrated lesions of the colorectum].

We reviewed 428 subjects with colorectal serrated lesions resected endoscopically or surgically at our institution. Colorectal serrated lesions were pathologically divided into 3 groups: hyperplastic polyp (HP), sessile serrated adenoma/polyp (SSA/P), and traditional serrated adenoma (TSA). SSA/P was detected frequently in the right colon and SSA/P was mainly flat-elevated. Cancers occurring in SSA/P were found more frequently than HP or TSA. The incidence of cancer in SSA/P was equivalent to that of cancer in traditional adenoma. Further studies are warranted to clarify clinicopathological features of serrated lesions of the colorectum.

A novel self-assembling peptide hemostatic gel as an option for initial hemostasis in endoscopic sphincterotomy-related hemorrhage: a case series.

Endoscopic sphincterotomy (EST) is a fundamental procedure of therapeutic endoscopic retrograde cholangiopancreatography, with post-EST bleeding as a serious adverse event. Although there are various hemostatic methods for post-EST bleeding, there is no consensus regarding the treatment choice. PuraStat is a novel self-assembling peptide developed as a hemostatic agent. We report six cases of EST-related hemorrhage with initial hemostasis achieved using PuraStat. The cases were observed in four men and two women, with an average age of 77.8 years. EST was performed for biliary drainage in four cases and for stone removal in two cases. Bleeding occurred during the same session as EST in five of six cases, with the remaining case showing bleeding 4 days after EST. As all patients with EST-related hemorrhage presented oozing with stable vital signs, we selected PuraStat as first-line hemostasis in each case. We applied PuraStat using a dedicated catheter with the tip pressed against the bleeding point. Hemostasis was confirmed without additional procedure in all cases. No adverse events were noted after the procedures. As PuraStat hemostasis is effective, feasible, and safe for EST-related hemorrhage, PuraStat may be an option for initial hemostasis, although it is limited to oozing.

Observation of the drainage process of the residual lipoma after endoscopic unroofing technique during colonoscopic evaluation of post-procedural hematochezia.

A 57-year-old man was referred to our hospital for further management of a subepithelial lesion noted on colonoscopy. He underwent endoscopic treatment of unroofing technique, in which the protruding portion of the tumor was partially resected. Due to a small amount of hematochezia, colonoscopy was performed to re-evaluate the lesion post-treatment. This enabled the observation of the drainage process of the residual lipoma. Remission was achieved and confirmed 8 months after the treatment. Endoscopic unroofing technique has been reported as a safe and effective method of treating lipomas, particularly large ones. To the best of our knowledge, this is the first endoscopic unroofing case in which the drainage process of the residual lipoma was observed and the remission of the lesion was confirmed.

Role of Serum Proteinase 3 Antineutrophil Cytoplasmic Antibodies in the Diagnosis, Evaluation of Disease Severity, and Clinical Course of Ulcerative Colitis.

BACKGROUND/AIMS: Proteinase 3 antineutrophil cytoplasmic antibody (PR3-ANCA) is a serologic marker for granulomatosis with polyangiitis. However, recent studies have also shown their role as diagnostic markers for ulcerative colitis (UC). This study was performed to investigate the clinical roles of PR3-ANCAs in the disease severity, disease extension, and clinical course of UC. METHODS: Serum PR3-ANCAs were measured in 173 UC patients including 77 patients with new-onset patients UC diagnosed within 1 month, 110 patients with Crohn's disease, 48 patients with other intestinal diseases, and 71 healthy controls. Associations between the PR3-ANCA titer and clinical data, such as disease severity, disease extension, and clinical course, were assessed. The clinical utility of PR3-ANCA measurement was evaluated by receiver operating characteristic (ROC) analysis. RESULTS: PR3-ANCA ≥3.5 U/mL demonstrated 44.5% sensitivity and 95.6% specificity for the diagnosis of UC in all patients. PR3-ANCA positivity was more prevalent in the 77 new-onset UC patients (58.4%). In this group, the disease severity and extension were more severe in PR3-ANCA positive patients than in PR3-ANCA negative group (p<0.001). After treatment, the partial Mayo scores were significantly decreased with the PR3-ANCA titers. The proportion of patients who required steroids for induction therapy was significantly higher among PR3-ANCA positive than negative group. ROC analysis revealed that PR3-ANCA ≥3.5 U/mL had 75% sensitivity and 69.0% specificity for steroid requirement in new-onset UC patients. CONCLUSIONS: Our results indicate that PR3-ANCA measurement is useful not only for diagnosing UC but also for evaluating disease severity and extension and predicting the clinical course.

Epstein-Barr Virus-positive Intestinal Diffuse Large B-cell Lymphoma in a Japanese Patient with Celiac Disease: First Reported Case and a Literature Review.

A 60-year-old Japanese woman was diagnosed with celiac disease (CeD) and treated with a gluten-free diet. For five years, she had a good clinical course. However, she complained of inappetence and nausea. Colonoscopy revealed ulcerative tumors in the terminal ileum. A histological examination of biopsy specimens from the ulcerative tumor showed diffuse infiltration of large atypical lymphocytes. Immunohistologically, the atypical lymphoid cells were positive for cluster of differentiation (CD) 10 and CD20. Many Epstein-Barr virus-encoded small RNA (EBER)-positive atypical lymphocytes were detected by in situ hybridization. This represents the first reported case of Epstein-Barr virus-positive intestinal diffuse large B-cell lymphoma complicated with CeD.

Endoscopic and clinicopathological characteristics of colorectal T/NK cell lymphoma.

BACKGROUND: Colorectal T/natural killer (NK)-cell lymphomas (TNKCL) are very rare. Endoscopic and clinicopathological characteristics of colorectal TNKCL have not been clearly demonstrated. In this study, we demonstrated the clinical characteristics of colorectal TNKCL. METHODS: Endoscopic and clinicopathological characteristics were investigated in 27 patients with colorectal monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL), adult T-cell leukemia/lymphoma (ATLL), and other types of TNKCL. RESULTS: Nine TNKCL patients (33%) were classified as MEITL, 11 (41%) as ATLL, and seven (26%) as other. Four patients with Epstein-Barr Virus-positive (EBV+) TNKCL, two indolent T-cell lymphoproliferative disorder and one anaplastic large cell lymphoma were included in the other group. Endoscopically, six MEITL (67%) and five ATLL (46%) showed diffuse-infiltrating type, in which the main endoscopic lesion was edematous mucosa in MEITL, while aphthoid erosion and edematous mucosa were typical in ATLL. Ulcerative type was identified in four other group patients (57%), including two EBV+ TNKCL. An increase in atypical T-intraepithelial lymphocytes (T-IELs) was noted in seven MEITL (88%) and six ATLL (60%) patients, but not in the other group (0%) patients. Five MEITL patients (56%) showed features of lymphocytic proctocolitis with increased CD8+ T-IELs. CONCLUSIONS: MEITL and ATLL occasionally invaded the colorectum, and primary involving MEITL was observed. Diffuse infiltrating type was the characteristic endoscopic finding in colorectal MEITL and ATLL, while ulcerative type was observed in the other group. Features of lymphocytic proctocolitis may be prodromal findings of MEITL.

Endoscopic and clinicopathological characteristics of gastrointestinal adult T-cell leukemia/lymphoma.

BACKGROUND: Adult T-cell leukemia/lymphoma (ATLL) frequently involves the gastrointestinal (GI) tract, and patients mainly show an aggressive clinical course despite of intensive cytotoxic treatments. We investigated the characteristic clinicopathological and endoscopic features of GI ATLL. METHODS: This was a retrospective analysis of 61 GI tract lesions in 54 ATLL patients. RESULTS: Thirty-six (67%) patients were classified as having lymphoma-type ATLL and 18 (33%) patients were classified as having acute-type with leukemic changes. Examined ATLL lesions in the stomach and intestine (small intestine and colorectum) were 40 (66%) and 21 (34%), respectively. Gastric ATLL lesions were frequently found in the lymphoma-type (29/38; 76%) compared with the acute-type lesions (11/23; 48%; P=0.023). Intestinal ATLL lesions were frequent in the acute-type (12/23; 52%) compared with the lymphoma-type lesions (9/38; 24%; P=0.023). Endoscopically, tumor-forming type lesions were significantly more frequent in lymphoma-type ATLL lesions (29/38 lesions; 76%) compared with acute-type lesions (10/23; 44%; P=0.0096). The superficial spreading-type was significantly more frequent in acute-type lesions (12/23 lesions; 52%) compared with lymphoma-type lesions (3/38; 8%; P=0.0003). Additionally, gastropathy-, enteropathy-, or proctocolitis-like lesions were distinct features, mainly in the acute type (9/23 lesions; 39%). Twenty three of 39 tumor-forming-type lesions (59%) were significantly composed of pleomorphic or anaplastic large cell lymphoma, and 13 of 15 superficial spreading-type lesions (87%) were significantly composed of pleomorphic medium-sized cells (P=0.007, in each). Six patients (11%) who were estimated as having primary GI ATLL based on restricted clinical stages, showed a significantly better overall survival (OS) compared with the 48 advanced-stage patients (P=0.017). Twenty patients with solitary tumor-forming-type lesions showed a significantly better OS than 17 patients with the multiple tumor-forming-type (P=0.015) and five with the mucosal-thickening-type lesions (P=0.04). Twenty-six patients with pleomorphic or anaplastic large cell ATLL showed a significantly better prognosis compared with 28 patients with pleomorphic medium-sized ATLL (P=0.034). CONCLUSIONS: ATLL predominantly involves the stomach. Leukemic behavior of ATLL had a large influence on the tumor location and endoscopic features of GI tract lesions. Gastropathy-, enteropathy-, and proctocolitis-like lesions showed additional distinct characteristics. Primary GI ATLL in the early clinical stages, solitary tumor-forming-type lesions and large tumor cells showed better prognostic factors than other factors, respectively.

Multiple lesions of gastrointestinal tract invasion by monomorphic epitheliotropic intestinal T-cell lymphoma, accompanied by duodenal and intestinal enteropathy-like lesions and microscopic lymphocytic proctocolitis: a case series.

BACKGROUND: In East Asia, monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL), previously known as type II enteropathy-associated T-cell lymphoma (EATL), occurs more frequently than type I EATL, and coeliac disease is rare. CASE PRESENTATION: Here we present four cases of MEITL in Japanese patients, including the endoscopic and pathological findings of their duodenal and colorectal lesions. Tumor specimens obtained from duodenal, intestinal, and colorectal biopsies in all four patients showed a diffuse intramucosal infiltration of small to/or medium-sized lymphoma cells and numerous atypical intraepithelial lymphocytes (IELs). These cells were immunohistologically positive for CD103, CD3, CD7, CD8, CD56, and T-cell intracellular antigen-1. Upper and lower gastrointestinal and antegrade double-balloon endoscopy revealed foci of edematous mucosa, with or without villous atrophy, in the non-neoplastic mucosa. Histological studies demonstrated duodenal and intestinal enteropathy-like lesions as well as microscopic (lymphocytic) proctocolitis with increased CD3- and CD8-positive and CD56-negative T-IELs in all four patients. The clinicopathological findings of the non-neoplastic lesions were similar to those characteristic of coeliac disease, suggesting that variants of coeliac disease may be present in the prodromal lesions of MEITL. CONCLUSIONS: Our study supports the need for random gastrointestinal biopsies to determine tumor spread, the features of MEITL in the particular patients, and the presence of prodromal non-neoplastic lesions.

High Expression of Intestinal Homing Receptor CD103 in Adult T-Cell Leukemia/Lymphoma, Similar to 2 Other CD8+ T-Cell Lymphomas.

We investigated the expression of the αEß7 integrin (CD103)-intestinal homing receptor of T-intraepithelial lymphocytes (IELs) in 130 cases of adult T-cell leukemia/lymphoma (ATLL). We detected CD103 lymphoma cells in 55% (31/56) of mainly gastrointestinal (GI)-involved ATLL cases. Among them, lymphoma cells of 18 cases located in other involved organs had similar CD103 expression patterns. Histologically, we found (a) increased reactive IELs in non-neoplastic mucosal layers in 28% (5/18) of surgical and mucosal resection cases, (b) preserved epithelial glands, and (c) numerous small intraepithelial ATLL nests in involved lesions in 36 (69%) and 21 (40%), respectively, of the 52 examined cases. These 3 patterns were common in intestinal type II enteropathy-associated T-cell lymphoma but were rare in intestinal EBV nasal-type/like T/natural killer (NK)-cell lymphoma. We detected CD103 tumor cells in 41% (16/39) of lymph node-involved ATLL, in 31% (11/35) of skin-involved ATLL, in 68% (21/31) of type II CD4 enteropathy-associated T-cell lymphoma cases, in 36% (8/22) of primary gastric T/NK-cell lymphomas, and in 77% (7/9) of CD8 epidermotropic mycosis fungoides. CD103 ATLL prefers involving the GI tract over the skin (P<0.05). CD103 expression in GI-involved and/or total ATLL cases was significantly higher than in other 9 T/NK-cell lymphoma groups (P<0.05 or 0.01). Only ATLL cases were commonly CD103 in CD4 T/NK-cell lymphoma groups (P<0.05 or 0.01). Human T-lymphotropic virus-1-infected CD103 T-IELs and mucosal T cells may be important sources of ATLL.

Endoscopic removal of a tablespoon lodged within the duodenum.

Here we report the case of a 34-year-old man who underwent endoscopic removal of a tablespoon from the stomach that was lodged within the duodenum. Removal required the use of a two-channel upper endoscope and polypectomy snares. Using the double-snare technique, the spoon was grasped at the proximal and distal parts of the handle. The double-snare was first pulled unsuccessfully and then pulled with simultaneous manual abdominal compression of the bulbus from the body surface. Compression was gently applied towards the stomach. As a result, the head of the spoon prolapsed from the bulbus, and was easily retracted from the stomach without any complications. In cases of foreign body lodging within the duodenum, the manual abdominal compression technique may help clinicians pull out the object and avoid surgery. The usefulness of manual compression is dependent on the foreign body's sharpness and the location.

Endoscopic ultrasonography of duodenal aberrant pancreas: comparison with histology after endoscopic resection.

We present the case of a 45-year-old man with an aberrant pancreas in the duodenum. He was referred to our hospital for gastric cancer screening. On upper gastrointestinal endoscopy, a submucosal tumor was noted in the second portion of the duodenum; it was 10 mm in diameter, with a smooth surface and bridging fold. Endoscopic ultrasonography (EUS) showed a hypoechoic lesion with small anechoic areas located in the third sonographic layer of the duodenum wall. To confirm the exact diagnosis, endoscopic resection was performed. The histological diagnosis was aberrant pancreas, Heinrich type II. The hypoechoic lesion and anechoic areas on EUS findings clearly corresponded with pancreatic acinus cells and duct dilation on histological findings, respectively. EUS findings are useful to diagnosis a duodenal aberrant pancreas that has ductal structures.

Colitis mimicking graft-versus-host disease during treatment with the anti-CCR4 monoclonal antibody, mogamulizumab.

A 57-year-old male with acute-type adult T cell leukemia-lymphoma (ATL) developed persistent watery, non-bloody diarrhea at a volume of 2-3 L/day following the administration of the anti-CC chemokine receptor 4 (CCR4) monoclonal antibody, mogamulizumab. An extensive examination revealed the absence of any pathogenic bacteria or parasites in his stool. Biopsied specimens from the colonic mucosa contained many small nests of apoptotic bodies in the colonic glands, which mimicked acute-colonic graft-versus-host disease. Activation of the auto-reactive immune system due to the depletion of regulatory T-cells by mogamulizumab was suspected as causative. Special attention should be paid to the risk of unique immune-related adverse events induced by mogamulizumab.