Plummer-Vinson syndrome: A decade's experience of 132 cases from a single center.

BACKGROUND AND AIM: Plummer-Vinson syndrome (PVS) comprises triad of iron deficiency anemia, dysphagia, and post-cricoid esophageal web. PVS is rare nowadays due to improved nutritional status. However, we encountered patients with PVS regularly at our center. Data regarding PVS are limited; hence, we aimed to study the clinical features, treatment outcomes, and development of complications in patients with PVS. METHODS: The study was conducted over a 10-year period (January 2008 to January 2018) in a medical college setting. All adults with dysphagia, anemia, and post-cricoid web or those with iron deficiency anemia and post-cricoids web were included in the study. Patients were treated with iron supplementation and Savary-Gilliard bougie dilation of the web. Patients were followed-up for the recurrence of dysphagia and development of complications. RESULTS: Overall, 153 patients exhibited esophageal web, of which 132 (86.27%) patients had concomitant PVS and 21 (13.7%) patients did not. The mean age was 43.50 years (range 16-76) and 113 (85.6%) were women. Single session of Savary-Gilliard bougie dilation was successful in 90.7% of patients in relieving dysphagia and 9.3% developed recurrence, requiring repeated dilations. Four patients had concomitant squamous cell carcinoma of esophagus along with PVS and two developed upper gastrointestinal malignancy during follow-up. CONCLUSION: Plummer-Vinson syndrome is predominantly seen in middle aged women and present with symptoms of iron deficiency anemia and early grade dysphagia. Single session of Savary-Gilliard bougie dilation was successful in majority of patients in relieving dysphagia. Overall risk of developing upper gastrointestinal malignancy was 4.5%.

Clinicopathological Correlation with Mesangial C4d Deposition in Primary Immunoglobulin A Nephropathy - A Descriptive study.

In immunoglobulin A (IgA) nephropathy, activation of lectin pathway leads to severe renal damage and more pronounced histological damage. As C4d is a marker of lectin pathway activation, the presence of mesangial C4d positivity will help in identifying those patients at risk. The study was conducted to study the prevalence of mesangial C4d positivity in patients with primary IgA nephropathy and to compare the clinical and histopathological features with C4d-positive and C4d-negative cases. It is a retrospective study conducted for four years. The inclusion criterion was IgA nephropathy with a minimum of four viable glomeruli. Biopsies with >25% of nonsclerotic glomeruli with mesangial positivity will be considered as positive for C4d. Seventy-six patients of IgA nephropathy were included of which mesangial C4d positivity was noted in 33 patients (43%). The mean age was 35 years. The male:female ratio was 2.3:1. The most common presentation was edema (56%) followed by microscopic hematuria (28%). Fifty-four patients were hypertensive. Among the clinical and laboratory parameters, absence of hematuria (P = 0.04) and presence of proteinuria (P = 0.02) showed a significant association with C4d positivity. The histological parameters in Oxford classification which had significant association with C4d positivity were segmental sclerosis (P = 0.01) and tubular atrophy (P = 0.001). Among 45 patients on follow-up with a maximum duration of 51 months, 10 developed end-stage renal disease of which four had C4d expression (0.05%) in the biopsy. Nearly half of IgA nephropathy patients have mesangial C4d positivity. Elevated creatinine with chronicity changes is more common in C4d-positive patients. Hence, C4d can be used as a marker for poor prognosis.

Lessons of the month: Massive gastrointestinal bleeding in a young woman with idiopathic thrombocytopenic purpura (ITP).

Cytomegalovirus (CMV) is a ubiquitous pathogen, belongs to the herpes virus family and can infect the gastrointestinal (GI) system. The disease is usually noted in immunocompromised patients such as solid organ transplant recipients on immunosuppressive drugs, patients with malignancy receiving chemotherapy, patients with AIDS, patients on steroids for autoimmune disorders, and is rarely seen in immunocompetent individuals. In the GI system, CMV most commonly involves the colon, followed by oesophagus, stomach and, rarely, the small intestine. The GI manifestation of CMV infection is usually anorexia, diarrhoea, and blood in stools, abdominal pain and fever. Very rarely, CMV infection may present with a massive GI bleed. We report a case of 36-year-old pregnant woman with idiopathic thrombocytopenic purpura (ITP) who presented with massive GI bleeding following delivery, attributed to isolated CMV enteritis.

Primary Intestinal Lymphoma: Clinicopathological Characteristics of 55 Patients.

INTRODUCTION: Gastrointestinal (GI) tract is the most common site of extranodal lymphoma accounting for 30-40% of the cases. In Western countries, stomach is the most common site of GI lymphoma, whereas in the Middle East and Mediterranean countries, small intestine is commonly involved. Studies about primary intestinal lymphoma (PIL) are heterogeneous in anatomical distribution, presentation, and histological subtypes. The present study was aimed at studying the anatomical distribution, histological subtypes, and clinical characteristics at tertiary care centers. MATERIALS AND METHODS: The present study was retrospective, conducted between 2006 and 2020. Patient's data were collected from institutional medical records. PIL was diagnosed by Lewin's criteria. After histological diagnosis, PIL was classified as per the World Health Organization (WHO) criteria and staging was done according to the Ann Arbor classification as modified by Musshoff. RESULTS: A total of 941 lymphoma cases were diagnosed during the study period between 2006 and 2020 consisting of 238 Hodgkin's lymphoma and 703 non-Hodgkin's lymphoma (NHL) cases. PIL constituted 5.8% of all lymphoma cases (55 out of 941) and 50.9% (55 of 108) of all primary GI lymphoma. Median age at diagnosis was 44 years and comprised predominantly males (85.45%). Diffuse large B-cell lymphoma (DLBCL) and mucosa-associated lymphoid tissue (MALT) lymphoma were the most common histological subtype (78%) seen. Two patients with primary Hodgkin's lymphoma involving the intestine were seen. T-cell lymphoma was seen in three (5.4%) patients. Ileocecal region was the most common site involved (27%). The common presenting complaints were intestinal obstruction (40%) requiring surgical resection and abdominal pain (32%). Majority of the patients presented in the early stages (I and II). CONCLUSION: Our study demonstrates the pattern of distribution and various histological subtypes of PIL including the rare variants like primary intestinal Hodgkin's lymphoma. Relatively more number of patients presented with intestinal obstruction requiring surgery in comparison with other studies. HOW TO CITE THIS ARTICLE: Malipatel R, Patil M, Rout P, et al. Primary Intestinal Lymphoma: Clinicopathological Characteristics of 55 Patients. Euroasian J Hepato-Gastroenterol 2021;11(2):71-75.

Primary Gastric Lymphoma: Clinicopathological Profile.

INTRODUCTION: Gastrointestinal tract (GIT) is the most common site of involvement of extranodal non-Hodgkin's lymphoma (NHL). There is regional variation in anatomical distribution of extranodal NHL, stomach being the most common site followed by small intestine. Primary gastric lymphoma (PGL) predominantly involves the antrum and corpus of the stomach. It arises from mucosa-associated lymphoid tissue (MALT) and is of B-cell lineage and often associated with Helicobacter pylori infection. Primary gastric lymphoma often presents with nonspecific symptoms. The present study was undertaken to ascertain the clinicopathological characteristics of PGL at a tertiary care center in South India. MATERIALS AND METHODS: It is a retrospective study from 2006 to 2016. Patient's data were obtained from institutional medical records. The histopathology slides were reviewed. The relevant immunohistochemistry (IHC) markers done were leukocyte common antigen (LCA), CD3, CD20, CD79a, CD10, Bcl-2, Bcl-6, CD5, Cyclin D1, CD138, and Ki-67. Correlating with the immunoprofile, further subtyping was done. RESULTS: A total of 405 patients of NHL were seen during the study period, out of which 43 patients were PGL. There were 32 males and 11 females, with M:F of 2.9:1. The mean age at diagnosis was 58 years. Abdominal pain and new-onset dyspepsia were the commonly observed presenting symptoms. The common site of involvement was antrum (20). Diffuse large B-cell lymphoma (DLBCL) was the most common histological subtype. Helicobacter pylori infection was seen in 18 (41%) patients. Majority of the patients were in stages II and III. CONCLUSION: In our study, the initial presentation of PGL was with nonspecific symptoms like abdominal pain and new-onset dyspepsia. High degree of suspicion of such symptoms and biopsy of all suspicious lesions is essential for early detection. Diffuse large B-cell lymphoma was the most common histological subtype seen in our study.How to cite this article: Malipatel R, Patil M, Rout P, Correa M, Devarbhavi H. Primary Gastric Lymphoma: Clinicopathological Profile. Euroasian J Hepato-Gastroenterol 2018;8(1):6-10.