Clinicopathological analysis of mucosa associated lymphoid tissue lymphoma secondary to Sjögren' s syndrome in salivary gland / 北京大学学报(医学版)

OBJECTIVE@#To analyze the clinicopathological characteristics of mucosa associated lymphoid tissue (MALT) lymphoma secondary to Sjögren' s syndrome (SS) (SS-MALT lymphoma) in salivary gland and to explore the value of the combined application of histopathological morphology, protein expression and molecular phenotype in pathological diagnosis and prognostic evaluation of SS-MALT lymphoma.@*METHODS@#Sixteen patients with SS-MALT lymphoma were collected from 260 patients who were diagnosed with SS in Peking University School and Hospital of Stomatology from January 1997 to December 2016. Twelve patients with non-MALT lymphoma secondary to SS (non-SS-MALT lymphoma) in salivary gland were selected as controls. The clinical data of the patients were retrospectively reviewed and analyzed. All the patients were followed up until December 20, 2019. Hematoxylin-eosin staining, immunohistochemistry, polymerase chain reaction (PCR) and fluorescence in situ hybridization (FISH) techniques were used to observe the histologic characteristics and to detect the manifestations of light chain restrictive expression, immunoglobulin (Ig) gene clonal rearrangement, chromosome translocation and gene abnormality, so as to evaluate their values in pathological diagnosis and prognostic evaluation.@*RESULTS@#The malignant transformation rate of SS to MALT lymphoma was about 6.15%, ranged from 3 to 240 months, during which 2 patients died due to high-level deterioration. Microscopically, the acini of the glandular tissue were atrophied and destroyed. The tumor cells dominated by central cell-like lymphocytes grew diffusely, destroying the epithelial islands. All SS-MALT lymphoma cases were positive in CD20 and Pax5. Half of them had the Ki-67 proliferation index of 10% or less, and half greater than 10%. 93.75% cases expressed AE1/AE3 protein, which showed the residual glandular epithelium. All the tumor cells were negative in CD3ε, and the plasma cells were detected by CD138 antigen. The light chain restrictive expression of κ and λ was 37.5% in SS-MALT lymphoma group. The positive detection rates of immunoglobulin heavy chain (IgH)-FR1, IgH-FR2, IgH-FR3, immunoglobulin kappa chain (IgK)-A, and IgK-B in SS-MALT lymphoma group were 33.3%, 53.3%, 33.3%, 20.0%, and 26.7%, respectively, and 93.3% when together used with IgH and IgK. The positive rates of the MALT1, IGH and BCL6 genes with dual color break-apart probes were 36.4%, 27.3% and 27.3%, and the detection rate of chromosome translocation and gene abnormality by applying the three probes was 72.7%.@*CONCLUSION@#There are no specific histological characteristics and protein phenotypes in the histologic diagnosis of SS-MALT lymphoma in salivary gland. The combined application of histopathological manifestations, immunohistochemistry, PCR and FISH techniques helps the accurate pathologic diagnosis of the disease. Although SS-MALT lymphoma is considered as an indolent lymphoma with a relatively favorable prognosis, the regular return visit and long-term follow-up should be conducted to detect the clues of recurrence and advanced deterioration.

Concurrent Gastric and Pulmonary Mucosa-Associated Lymphoid Tissue Lymphomas with Pre-Existing Intrinsic Chronic Inflammation: A Case Report and a Review of the Literature

Herein, we report a rare case of concurrent gastric and pulmonary mucosa-associated lymphoid tissue (MALT) lymphomas. A 65-year-old man who had been diagnosed with Helicobacter pylori-positive gastric MALT lymphoma received eradication therapy and achieved complete remission. During follow-up, he developed de novo pulmonary MALT lymphoma as a sequela of pulmonary tuberculosis, accompanied by recurrent gastric MALT lymphoma. Polymerase chain reaction (PCR) products of the CDR3 region of the immunoglobulin heavy chain gene showed an overall polyclonal pattern with bands at 400 base pairs (bp) and 200 bp predominant in the pulmonary tissue, as well as two distinctive bands in the gastric tissue at 400 bp and 200 bp. This case suggests that multiorgan lymphomas are more likely to be independent from each other when they are far apart, involve different organ systems, and have independent precipitating factors.

Concurrent Gastric and Pulmonary Mucosa-Associated Lymphoid Tissue Lymphomas with Pre-Existing Intrinsic Chronic Inflammation: A Case Report and a Review of the Literature

Herein, we report a rare case of concurrent gastric and pulmonary mucosa-associated lymphoid tissue (MALT) lymphomas. A 65-year-old man who had been diagnosed with Helicobacter pylori-positive gastric MALT lymphoma received eradication therapy and achieved complete remission. During follow-up, he developed de novo pulmonary MALT lymphoma as a sequela of pulmonary tuberculosis, accompanied by recurrent gastric MALT lymphoma. Polymerase chain reaction (PCR) products of the CDR3 region of the immunoglobulin heavy chain gene showed an overall polyclonal pattern with bands at 400 base pairs (bp) and 200 bp predominant in the pulmonary tissue, as well as two distinctive bands in the gastric tissue at 400 bp and 200 bp. This case suggests that multiorgan lymphomas are more likely to be independent from each other when they are far apart, involve different organ systems, and have independent precipitating factors.

Pulmonary manifestations of primary sjögren’s syndrome.

Sjögren's syndrome (SS) is a complex autoimmune exocrinopathy with multifactorial pathogenesis and multisystem manifestation. It is called primary Sjögren's syndrome (PSS) when the manifestations are seen without any other co-existent rheumatic diseases. The incidence of respiratory system involvement varies widely in the reported medical literature, partly due to lack of a universal agreement over the diagnostic criteria of the disease and the type of study methods employed. Respiratory system manifestations are protean; upper airway symptoms are very common and so is the complaint of dry cough. The PSS patients may develop interstitial lung diseases (ILDs) such as usual interstitial pneumonia (UIP), non-specific interstitial pneumonia (NSIP), lymphocytic interstitial pneumonia (LIP), bronchiolitis obliterans and organising pneumonia (BOOP), etc. They may also develop the whole spectrum of lymphoproliferative disorders of the lung ranging from LIP to follicular bronchiolitis, nodular lymphoid hyperplasia and low-grade lymphomas. Therapeutic options include symptomatic and supportive measures and corticosteroids as the mainstay of the treatment for ILDs occurring in these patients. In recent years, rituximab (anti-CD20) has emerged as a promising treatment for this disease, though data from controlled trials are still lacking. Pulmonary involvement may be a source of significant morbidity in these patients, though only rarely, it is the cause of death.

Linfoma MALT: Presentación de tres casos / MALT lymphoma: Presentation of three cases

El Helicobacter Pylori (HP) en una bacteria Gram negativa que crece en un medio microaerofílico, que se ha comprobado sea responsable de enfermedades del tracto gastrointestinal, específicamente el estómago, donde causa lesiones de la mucosa gástrica de diversa magnitud que va desde una gastritis aguda ligera hasta cuadros de mayor severidad como la úlcera gástrica y/o duodenal. También se ha visto asociado a dos tipos de tumores gástricos como adenocarcinoma de tipo intestinal, y linfoma linfoide asociado a la mucosa (linfoma Malt). En nuestro estudio se revisaron los casos de neoplasia gástrica en el período de 2 años encontrándose 3 casos de linfomas Malt detectándose esta entidad en estudios realizados como panendoscopias y toma de muestra para test de ureasa y estudio histológico, confirmándose así el diagnóstico. Dentro de los síntomas más frecuentes presentados se encontraban dolor en epigastrio, pérdida de peso y náuseas. Con estos resultados se indica una terapia erradicadora del Helicobacter Pylori con el objetivo de evitar otras complicaciones y eliminar las lesiones gástricas reportadas en el estudio realizado, además al paciente reportado con lesión de displasia de alto grado se le realizó tratamiento quirúrgico combinado con radioterapia y quimioterapia...

The Helicobacter Pylori (HP) is a Gram negative bacterium growing in microaerophilic medium that has been tested responsible for diseases of the gastrointestinal tract, mainly of the stomach, where it causes lesions of the gastric mucous of several magnitudes, leading from light acute gastritis to more severe diseases as gastric and/or duodenal ulcer. It has also been associated to two kinds of gastric tumors: adenocarcinoma of intestinal kind, and lymphoid lymphoma associated to mucosa (MALT lymphoma). In our study we reviewed all the cases of gastric neoplasias in a two-year period, finding 3 cases of MALT lymphomas, detected in such studies like panendoscopy to take samples for ureasa testing, and histological studies. That way the diagnosis was confirmed. Among the most frequent symptoms were epigastria pain, weight lost and nauseas. With these results, a therapy was indicated to eradicate the Helicobacter Pylori to avoid other complications and to eliminate the reported in the study gastric lesions. Besides that, the patient reported with a high degree dysplasia lesion was operated and received radiotherapy and chemotherapy.

Helicobacter heilmannii-associated Gastritis: Clinicopathologic Findings and Comparison with Helicobacter pylori-associated Gastritis

The aims of this study were to evaluate the clinicopathologic features of Helicobacter heilmannii-associated gastritis and to compare H. heilmannii-associated gastritis with H. pylori-associated gastritis. We reviewed 5,985 consecutive gastric biopsy specimens. All cases of chronic gastritis with Helicobacter infection were evaluated with the Updated Sydney System, and the grades of all gastritis variables were compared between H. heilmannii-associated gastritis and H. pylori-associated gastritis groups. There were 10 cases of H. heilmannii-associated gastritis (0.17%) and 3,285 cases of H. pylori-associated gastritis (54.9%). The organisms were superficially located within the mucous layer without adhesion to epithelial cells. Interestingly, in one case many intracytoplasmic H. heilmannii organisms were observed in parietal cells with cell damage. A case of low-grade mucosa-associated lymphoid tissue (MALT) lymphoma concomitant with H. heilmannii infection was detected. Compared to H. pylori-associated gastritis, H. heilmannii-associated gastritis showed less severe neutrophilic activity (p<0.0001), mononuclear cell infiltration (p=0.0029), and endoscopic findings of chronic gastritis devoid of erosion or ulcer (p=0.0309). In conclusion, we present the detailed clinicopathologic findings of H. heilmanniiassociated gastritis compared to H. pylori-associated gastritis. H. heilmannii-associated gastritis is uncommon and milder than H. pylori-associated gastritis, however it may be noteworthy with respect to the development of MALT lymphoma.

[New advances in the management of gastric marginal zone B cell lymphoma]

The stomach is the most common site involved in primary gastrointestinal lymphoma. Helicobacter pylon [HP] plays a decisive role in the pathogenesis of gastric marginal zone B cell lymphoma of mucosa associated lymphoid tissue. Eradication therapy has become widely accepted as initial treatment of localized low grade MALT lymphoma. Further studies are needed to determine the most adapted treatment in non-responding patients. A long-term endoscopic follow-up is recommended due to the increased risk of gastric adenocarcinoma in spite of Helicobacter pylon eradication

Is the presence or absence of Helicobacter pylori in gastric mucosa a greater risk?

Helicobacter pylori is a unique organism which is pathogenic for stomach-duodenum (chronic gastritis, duodenal ulcer, gastric ulcer, gastric malignancy, mucosa-associated lymphoid tissue (MALT) lymphoma) and protective for oesophagus (Barrett's oesophagus, oesophageal adenocarcinoma) at the same time in an individual. For prevention of diseases, the necessity of presence of some bacteria throughout the gastrointestinal lumen needs to be emphasized. The concept--only good Helicobacter pylori is a dead Helicobacter pylori, is dangerous and humans should learn to live in harmony with a few bacteria throughout the gastrointestinal tract.

Primary gastric lymphoma and Helicobacter pylori infection with gastric amyloidosis.

Primary gastric lymphoma, an uncommon gastric tumor caused by infection with Helicobacter pylori, is rarely associated with gastric amyloidosis. Chronic bacterial infection is known to cause amyloidosis. We report a 53-year-old man who had an antral and duodenal mass with narrowing and ulceration on endoscopy and CT scan; endoscopic biopsy revealed gastric amyloidosis. Rapid urease test and serology for H. pylori were positive. Histology of resected specimen of distal stomach revealed primary gastric lymphoma, amyloid deposits and spiral organisms suggestive of H. pylori. Rectal biopsy was negative for amyloid. He remained well on follow-up after surgery and eradication of H. pylori.

Manifestações de linfoma na síndrome de Sjõgren: existe relação?

A Síndrome de Sjõgren (SS) é considerada uma afecção multissistêmica, crônica, que se caracteriza pela infiltração linfocítica nas glândulas exócrinas e a produção de auto-anticorpos. OBJETIVO: Vários estudos têm notado um aumento na incidência de linfomas malignos em pacientes com SS. Em nosso estudo tentamos descrever esta relação. FORMA DE ESTUDO: Coorte transversal. MATERIAL E MÉTODO: Pacientes com Síndrome Sicca acompanhados no ambulatório de Estomatologia do Departamento de Otorrinolaringologia da Santa Casa de Misericórdia de São Paulo, no período de julho de 1999 a abril de 2002. RESULTADOS: Dos 39 pacientes, 24 foram classificados com SS. A idade variou de 19 a 83 anos, com predominância do sexo feminino (69,7 por cento). O intervalo de tempo entre o início dos sintomas e o diagnóstico de SS variou de 3,77 anos. Não foi observado desenvolvimento de linfoma em nenhum dos pacientes avaliados. CONCLUSÃO: O diagnóstico de SS e o aumento do risco de desenvolvimento de Linfoma ao longo dos anos são importantes, então um longo período de seguimento destes pacientes é fundamental. Observamos que nossos achados foram diferentes quando comparados com a literatura. Nós não encontramos nenhum linfoma em nossos pacientes.

Dolor abdominal, dispepsia y gastritis en pediatría: rol del helicobacter pylori / Abdominal pain, dyspepsia and gastritis in paediatrics: role of the helicobacter pylori

Helicobacter pylori (H. pylori), el patógeno más común del tracto gastrointestinal en seres humanos, es la causa más frecuente de gastritis crónica, está asociado etiológicamente con úlcera gastroduodenal y algunos cánceres gástricos. La creciente acumulación de información ha hecho necesario reevaluar los conceptos de gastritis, dolor abdominal y dispepsia en pediatría, por lo que el objetivo de esta revisión fue actualizar estos conceptos enfatizando la relación de estas relación de estas entidades y discutir la evidencia disponible para promover una buena práctica clínica, al respecto. La asociación entre la infección por H pylori y otras condiciones, como dolor abdominal recurrente y dispepsia no ulcerosa, no ha sido demostrada y es más bien especulativa. El H. pylori es considerado un patógeno, porque, entre otras características, cumple cada uno de los postulados de Koch como causante de gastritis crónica; en efecto, H. pylori juega un rol crítico y necesario en la patogénesis de la gastritis crónica activa. El papel H. pylori como causa de úlcera péptica en adultos ha sido bien establecido. Aunque en niños las evidencias no son tan potentes, sí son altamente sugerentes. aun cuando la mayoría de la población mundial está colonizada por H. pylori, solo una pequeña proporción tendrá manifestaciones clínicas producidas por la infección. Aproximadamente el 10 por ciento de ellos desarrollará úlcera péptica a lo largo de su vida, y las personas infectadas tendrán un incremento de 2 a 6 veces en el riesgo de desarrollar cáncer y lifoma tipo MALT (tejido linfoide asociado a mucosas) comparado con población no infectada

Non-Hodgkin's lymphoma & primary biliary cirrhosis with Sjogren's syndrome

Sjogren's syndrome (SS) is an autoimmune disease characterized by a lymphocytic infiltration of the salivary and lacrimal glands leading to a progressive destruction of these glands due to the production of autoantibodies. This disorder is either isolated (primary SS) or associated with other systemic diseases (secondary SS). The occurrence of B-cell non-Hodgkin's lymphoma (NHL) represents the major complication in the evolution of SS patients. The risk of developing NHL, which is equivalent for both primary and secondary SS, was estimated to be 44 times greater than that observed in a comparable normal population. NHLs in SS patients occur preferentially in the salivary glands and in other mucosa-associated lymphoid tissues (MALT). However, it can also occur in the lymph nodes or bone marrow. We documented a case of low-grade B-cell lymphoma of MALT in the right eyelid and primary biliary cirrhosis (PBC) of a patient with SS. To the best of our knowledge, this is the first case reported in Korea.

Helicobacter pylori and Iymphoid follicles in primary gastric MALT-Iymphoma in Mexico

Los linfomas gástricos primarios de tipo MALT se han asociado con la infección por Helicobacter pylori. En el Instituto Nacional de Cancerología de la ciudad de México se revisaron en un periodo de 12 años (1982-94) los casos de linfoma gástrico primario MALT. Se buscaron en la mucosa gástrica adyacente a la neoplasia, cambios morfológicos relacionados con la infección: microorganismos morfológicamente compatibles con H pylori, folículos linfoides y gastritis crónica activa. Hubo 23 casos (85 por ciento) de linfomas MALT de grado bajo y cuatro (15 por ciento) de grado alto. En 23 de los 27 casos (85 por ciento, IC 95 por ciento = 72-99 por ciento se identificó H pylori; en 15 de los 23 (65 por ciento IC = 56-74 por ciento) hubo folículo linfoides. En 25 de los 27 casos (93 por ciento, IC = 90-95 por ciento) se encontró gastritis crónica activa. Hubo una asociación estrecha entre la infección por H pylori y el linfoma gástrico primario de tipo MALT. La presencia de folículos linfoides mostró ser un indicador confiable de la infección, además de ser el sustrato fisiopatológico propuesto para los linfomas MALT. Los pacientes en quienes se observaron folículos linfoides deben ser vigilados en forma periódica ya que H pylori es probablemente promotor de la transformación neoplásica