Intestinal Mucosa-associated Lymphoid Tissue Lymphoma Research Articles

Massive bleeding in small intestinal mucosa-associated lymphoid tissue lymphoma associated with COVID-19 infection: a case report

Abstract The increased risk of mucosa-associated lymphoid tissue (MALT) lymphoma is closely associated with chronic antigenic stimulation, with infection being the most common cause of recurrence. Lesions are usually associated with the gastrointestinal tract, and the involvement of small intestinal is rare. Recent studies have established a close relationship between novel coronavirus 2019 (COVID-19) and the occurrence and progression of various diseases. This article presents a rare case of a small intestinal MALT lymphoma. The patient was initially admitted with COVID-19 pneumonia and subsequently developed gastrointestinal bleeding during hospitalization. Medical and endoscopic treatments were ineffective, and an emergency exploratory laparotomy was performed. The affected segment of the small intestine was excised, and a pathological biopsy confirmed the diagnosis of MALT lymphoma. This case underscores the significance of raising clinical awareness of this condition among health care professionals.

Mucosa-Associated Lymphoid Tissue (MALT) Lymphoma in the Gastrointestinal Tract in the Modern Era.

Simple SummaryExtranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma), which is frequently linked to chronic antigenic stimulation, is clinically an indolent B-cell lymphoma. The most common site is the stomach, accounting for 35% of MALT lymphomas, with a strong association with Helicobacter pylori (H. pylori) infection. Although antibiotic eradication is first-line therapy for gastric MALT lymphoma, a recent trend showing increasing H. pylori-negative cases may require different treatment strategies. Intestinal MALT lymphoma, including immunoproliferative small intestinal disease, is relatively rare, and the pathogenesis and therapeutic approach have not been fully elucidated. In addition, the gastrointestinal tract was recently considered a preferable site of Epstein–Barr-virus-negative marginal zone lymphoma in the post-transplant setting. We review the updated clinicopathological features, treatment, and evolving concepts of MALT lymphoma of the gastrointestinal tract.Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) typically arises from sites such as the stomach, where there is no organized lymphoid tissue. Close associations between Helicobacter pylori and gastric MALT lymphoma or Campylobacter jejuni and immunoproliferative small intestinal disease (IPSID) have been established. A subset of tumors is associated with chromosomal rearrangement and/or genetic alterations. This disease often presents as localized disease, requiring diverse treatment approaches, from antibiotic therapy to radiotherapy and immunochemotherapy. Eradication therapy for H. pylori effectively cures gastric MALT lymphoma in most patients. However, treatment strategies for H. pylori-negative gastric MALT lymphoma are still challenging. In addition, the effectiveness of antibiotic therapy has been controversial in intestinal MALT lymphoma, except for IPSID. Endoscopic treatment has been noted to usually achieve complete remission in endoscopically resectable colorectal MALT lymphoma with localized disease. MALT lymphoma has been excluded from post-transplant lymphoproliferative disorders with the exception of Epstein–Barr virus (EBV)-positive marginal zone lymphoma (MZL). We also describe the expanding spectrum of EBV-negative MZL and a close association of the disease with the gastrointestinal tract.

Small intestinal mucosa-associated lymphoid tissue lymphoma with deep ulcer and severe stenosis: A case report.

IntroductionAlthough eosinophils are commonly present on the mucosa of the gastrointestinal tract, various pathological conditions may cause a secondary increase in eosinophil quantity.Presentation of caseA 78-year-old man was referred to our hospital due to abdominal pain. Examinations revealed an ulcerative lesion with white moss in the terminal ileum and severe stenosis on the oral and anal sides. Tissue biopsies obtained from the ulcer margins showed a predominance of chronic inflammatory cells and abundant eosinophils in addition to lymphocytes/plasma cells. Secondary causes of tissue eosinophilia were suspected; however, the diagnosis could not be confirmed because of atypical endoscopic findings. Partial resection of the ileum was performed for therapeutic and diagnostic purposes. Histopathology of the resected specimen identified a lymphoepithelial lesion with an invasive tendency. While CD20 staining was positive, MUM-1 and Bcl-6 staining were negative. Based on these findings, the lesion was diagnosed as a small intestinal mucosa-associated lymphoid tissue lymphoma (Lugano staging, stage II1).DiscussionHypereosinophilia in this lesion was suggested to be secondary to chronic inflammation due to tumor growth or impaired transit.ConclusionThere is a type of gastrointestinal MALT lymphoma showing an invasive tendency. In such cases, it may demonstrate atypical findings and hypereosinophilia in gastrointestinal tissues.

Radiotherapy for non-gastric intestinal versus gastric MALT lymphoma: a comparison of treatment outcomes.

BackgroundRadiotherapy is often used for treating patients with gastric mucosa-associated lymphoid tissue (MALT) lymphomas who fail to respond to Helicobacter pylori eradication. However, non-gastric intestinal MALT lymphoma is rare, and no standard therapeutic strategies have been established. This study was designed to assess the long-term prognosis of non-gastric intestinal MALT lymphoma treated with radiotherapy and to compare the outcomes with that of post-radiotherapy gastric MALT lymphoma.MethodsThe study included 34 patients with stage I EA gastrointestinal MALT lymphoma according to the Ann Arbor classification who underwent definitive radiotherapy. The primary site was the rectum in 3, the duodenum in 1, and the stomach in 30 patients. The radiotherapy dose was 1.5‒2.0 Gy (median, 1.5 Gy) and the total dose was 30‒40 Gy (median, 30 Gy). The clinical target volume (CTV) was defined as the volume of the entire organ with the lymphoma. Adjacent lymph node areas were not routinely included in the CTV.ResultsComplete response (CR) was achieved in all patients. There were no local recurrences, and two cases of recurrence were observed at other sites. The 5-year overall survival rates for non-gastric and gastric MALT lymphomas were 100% and 94.7%, respectively, and the 5-year disease-free survival rates were 100% and 95.7%, respectively. None of the patients died of the current illness.ConclusionRadiotherapy for non-gastric intestinal MALT lymphoma is expected to result in good local control and long-term survival, similar to that for gastric MALT lymphoma.

MALT lymphoma of the small bowel with protein-losing enteropathy

Mucosa-associated lymphoid tissue (MALT) lymphoma usually arises from chronic inflammation. We herein report a case of small intestinal MALT lymphoma with protein-losing enteropathy (PLE). A 73-year-old woman presented with lower leg edema and severe hypoalbuminemia. She had a medical history of pylorus-preserving pancreaticoduodenectomy with Billroth II reconstruction. Oral and anal route double-balloon enteroscopies revealed irregular nodular mucosal lesions with erosion extending from the jejunum to terminal ileum. Histopathological evaluation of the biopsied mucosa showed proliferation of small-to-medium-sized lambda light chain-restricted B cells. Plasmacytic differentiation and lymphoepithelial lesions were present, leading to the diagnosis of MALT lymphoma. Tc-99m albumin scintigraphy indicated tracer exudation in the small bowel, suggesting the presence of PLE. Combination immunochemotherapy with rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisolone (R-CHOP) regimen improved both MALT lymphoma and PLE, whereas rituximab monotherapy was not successful. This case is considered to be common type of MALT lymphoma at an uncommon site and is distinct from immunoproliferative small intestinal disease (IPSID). To our knowledge, this is the first case of non-IPSID-type small intestinal MALT lymphoma complicated by PLE. Gastrointestinal reconstruction may be responsible for underlying chronic inflammation via small intestinal bacterial overgrowth.

Expression of PIK3CA and FOXP1 in gastric and intestinal non-Hodgkin’s lymphoma of mucosa-associated lymphoid tissue type

Non-Hodgkin's lymphoma of mucosa-associated lymphoid tissue (MALT) type arises from a wide variety of extranodal sites, most frequently from the gastrointestinal tract. Recently, it has been demonstrated that karyotypic alterations involving the PIK3CA and FOXP1 genes of chromosome 3 occur in MALT lymphoma. However, their associated protein expression has not been extensively studied. Tumor tissues from 27 gastric and 23 intestinal MALT lymphomas were analyzed for PIK3CA and FOXP1 protein expression using immunohistochemistry and correlated with histological features and treatment outcomes. Expression of PIK3CA, a novel indicator, was found in 40% of gastrointestinal cases and indicated an inferior progression-free survival in both gastric and intestinal MALT lymphomas (P = 0.001 and P = 0.015). Tumor staining of nuclear FOXP1 (46.0%) was more common in gastric than intestinal MALT lymphomas (P = 0.042) and was significantly associated with polymorphic histology (P = 0.007). FOXP1 expression was identified as an adverse prognostic factor for overall survival in gastric MALT lymphomas (P = 0.035). We further combined these two markers and observed that patients that are positive for both PIK3CA and FOXP1 had a worse overall and progression-free survival. Considering the small sample size of this study, these results should be confirmed in a large prospective study.

Small Intestinal Mucosa Associated Lymphoid Tissue (MALT) Lymphoma in a Child Presenting with Recurrent Intussusception: A Case Report

Mucosa associated lymphoid tissue (MALT) lymphoma is a malignant B cell lymphoma that develops from MALT. The stomach is the most common site of MALT lymphomas but be found anywhere in the body. Adult MALT lymphomas are not rare diseases, but in children, intestinal MALT lymphomas are very rare. We treated a 12-year-old boy who underwent segmental resection of the small intestine due to recurrent intussusceptions, 2 months apart, and was diagnosed with an intestinal MALT lymphoma. He received multiple chemotherapeutic agents (vincristin, cyclophosphamide, prednisone, methotrexate, doxorubicin and cytosine arabinoside) according to the CCG-5961 protocol for 3 months. Currently, he

Association between Chlamydia psittaci infection and extranodal marginal zone B-cell lymphoma of mucosa associated lymphoid tissue (MALT)-lymphomas

7568 Background: MALT-lymphomas are often associated with infectious organisms. Microbial species associated with MALT lymphomas are: Helicobacter pylori (H.pylori), Campylobacter jejuni, Borrelia burgdorferi, and Hepatitis C Virus. Recent studies showed evidence of Chlamydia psittaci (C. psittaci) infection in 80% of ocular adnexal lymphoma and tumor regression following eradication therapy. We studied the presence of C. trachomatis, C. pneumoniae, and C. psittaci DNAs in MALT lymphomas of various sites and in non-malignant controls. Methods: MALT lymphomas from the following sites were selected for analysis: salivary gland (n=13), thyroid gland (n=4), skin (n=2), ocular adnexa (n=2), stomach (n=6), intestinum (n=4). Samples of Sjoegren syndrome (n=10) and samples of H. pylori positive gastritis (n=7) were included as non-malignant controls. Macrodissected tissue containing >80% lymphoma cells from paraffin embedded tissue was processed for DNA isolation. For the presence of C. psittaci and C. pneumoniae DNA real time PCR assays were used. For C. trachomatis a commercially available PCR assay (Roche Molecular Diagnostics) was employed. All samples were tested twice. A sample was regarded as positive if at least one positive result was obtained. Results: C. psittaci was found at variable frequencies in MALT lymphomas of different sites: 4/13 (31%) salivary gland, 2/4 (50%) thyroid gland, 2/2 (100%) skin, 2/2 (100%) ocular adnexa, 1/6 (17%) gastric, and 0/4 intestinal MALT lymphomas. Among the non-malignant specimens, C. psittaci DNA was detected in 4/10 (40%) samples of Sjoegren syndrome and in 0/7 samples of H. pylori positive gastritis. All nongastric MALT lymphomas tested were negative for C. trachomatis and C. pneumoniae. Conclusion: Our data reveal a clear association of C. psittaci infection with extragastric MALT lymphoma. However, no evidence could be established for C. trachomatis and C. pneumoniae infection. Moreover, the high prevalence of Sjoegren syndromes - the most frequent precursor lesion for MALT lymphomas of salivary gland and ocular adnexa - may suggest an involvement of C. psittaci induced antigenic driven lymphomagenesis in a significant proportion of extragastric MALT lymphomas. No significant financial relationships to disclose.

Mycosis fungoides associated with intestinal mucosa‐associated lymphoid tissue lymphoma

Coexistence of cutaneous T-cell lymphoma (CTCL) and a B-cell malignancy is rare. We report a case of mycosis fungoides (MF) associated with intestinal mucosa-associated lymphoid tissue (MALT) lymphoma, which is a low-grade B-cell lymphoma of marginal cell origin. To the best of our knowledge, there have been only two cases of coexistent CTCL and MALT lymphoma, and this is the first case with typical MF and intestinal MALT lymphoma. The coexistence in our case might be coincidental, but genetic predisposition and the immunoregulatory capacity of neoplastic T lymphocytes may be factors contributing to this association.

Mycosis fungoides associated with intestinal mucosa-associated lymphoid tissue lymphoma

Mycosis fungoides associated with intestinal mucosa-associated lymphoid tissue lymphoma

Chromosomal translocation t(11;18)(q21;q21) in gastrointestinal mucosa associated lymphoid tissue lymphoma

Aims: To evaluate the chromosomal translocation t(11;18)(q21;q21) in gastrointestinal lymphomas. Methods: A possible API2–MLT fusion transcript specific to t(11;18)(q21;q21) was examined by means of reverse transcription-polymerase chain reaction (RT-PCR) in...

Endoscopic diagnosis of ileocecal mucosa-associated lymphoid tissue lymphoma

Mucosa-associated lymphoid tissue lymphoma (MALT lymphoma) is a distinctive type of B-cell lymphoma that arises from mucosa-associated lymphoid tissue (MALT) in the GI tract, lung, salivary gland, and thyroid. 1 In the GI tract, MALT lymphomas occur most commonly in the stomach, whereas intestinal MALT lymphomas are rare. MALT lymphoma of the ileocecal valve has not been reported. We report here the endoscopic and clinicopathologic features of two cases of early MALT lymphoma that arose from the ileocecal valve.

Clinicopathologic study of primary intestinal B cell malignant lymphoma.

Primary intestinal B cell lymphoma is one of the most common extra-nodal lymphomas, which includes two types: intestinal mucosa-associated lymphoid tissue lym phoma ( IMALToma ) and lymphomatous polyposis ( LP ). Both have characteristic pathologic features, immunophen-otypes and biological behaviors. In this article, twenty-five cases were retrospectively analyzed with regard to criteria of diagnosis and clinicopathologic characteristics.

Intestinal Dissemination of Gastric Mucosa-Associated Lymphoid Tissue Lymphoma

Despite increasing identification of concurrent gastric and intestinal lymphomas of mucosa-associated lymphoid tissue (MALT), the clonal relationship between the two tumors and their sequential development are poorly understood. It is also unknown whether the development of these concurrent tumors is closely associated with direct antigen stimulation, which is thought to play an important role in the clonal expansion of low-grade MALT lymphomas. To investigate these, we have studied six cases of concurrent gastric and intestinal MALT lymphomas by polymerase chain reaction (PCR) amplification, cloning, and sequencing of the rearranged Ig gene, a strategy that has been widely used for analysis of clonality and antigen-driven properties of B-cell malignancies. In each case, an identical or nearly identical complementarity determining region (CDR) 3 sequence was observed between the dominant clones of concurrent gastric and intestinal MALT lymphomas. In four of six cases examined, sufficient Ig variable region sequence information was obtained to permit analysis of somatic mutations. The mutation patterns in one case suggest that the intestinal lesion is secondary to the gastric tumor, and the mutation patterns in two cases indicate that the gastric and intestinal lesions are derived from different tumour subclones, which emerge after expansion of a common early tumor clone. Furthermore, three of four cases showed ongoing Ig mutations among different PCR clones at each site. These results show that concurrent gastric and intestinal MALT lymphomas are derived from the same clone and suggest that the intestinal lesions result from dissemination of gastric tumours. Antigen stimulation may play a role in tumor evolution, particularly at an early stage.