Clinical Analysis of Pulmonary Lymphoma / 中国实验血液学杂志

OBJECTIVE@#To study the clinical, imaging, and pathological features of pulmonary lymphoma.@*METHODS@#Patients with pulmonary lymphoma diagnosed by lung biopsy in Zhongda Hospital Affiliated to Southeast University from November 2013 to December 2020 were collected and divided into secondary pulmonary lymphoma (SPL) group and primary pulmonary lymphoma (PPL) group according to the primary site of lymphoma. The clinical characteristics, stages, imaging features, diagnostic methods and pathological types of the two groups were analyzed.@*RESULTS@#A total of 22 patients were included, 10 cases were PPL and 12 cases were SPL. The main symptoms of the two groups were cough, dyspnea and chest pain. The proportion of stage III/IV patients and international prognostic index (IPI) in SPL group were significantly higher than those in PPL group (P<0.05). Chest high-resolution computed tomography (HRCT) mainly showed masses, nodules and consolidation in both groups. The proportions of single mass and air bronchial sign in PPL group were significantly higher than those in SPL group, while the proportions of multiple nodules, mediastinal/hilar lymphadenopathy and pleural effusion were significantly lower (P<0.05). The max standardized uptake value (SUVmax), peak standardized uptake value (SUVpeak), total lesion glycolysis (TLG) and metabolic tumor volume (MTV) of 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography/computed tomography (PET/CT) in PPL group were lower than those in SPL group, but the differences were not statistically significant (P>0.05). In PPL group, 8 cases were diagnosed by transbronchial lung biopsy (TBLB) and 2 cases by percutaneous lung puncture. In SPL group, 4 cases were diagnosed by TBLB, 7 cases by percutaneous lung puncture, and 1 case by surgery. 95.5% patients were diagnosed by non-surgical methods. The main pathological type of PPL was mucosa-associated lymphoid tissue (MALT) lymphoma, while that of SPL was diffuse large B-cell lymphoma (P<0.05).@*CONCLUSION@#The clinical symptoms of pulmonary lymphoma are nonspecific, but the chest HRCT has characteristic manifestations, which can also help to distinguish between SPL and PPL. 18F-FDG PET/CT is also a potential method to distinguish between SPL and PPL. TBLB and percutaneous lung puncture biopsy are reliable methods for the diagnosis of lung lymphoma. The main pathological type of PPL is MALT lymphoma, while that of SPL is diffuse large B-cell lymphoma.

Atypical presentation of Sjogren's syndrome with MALT lymphoma mimicking chronic venous ulcers / Apresentação atípica da síndrome de Sjogren com linfoma MALT que mimetiza úlceras crônicas venosas

Abstract Chronic ulcerations of the lower extremities are quite a common condition amongst adults, most often caused by chronic venous insufficiency. Irrespective of the main underlying cause, chronic limb ulcerations are usually associated with significant symptoms, impairing daily functioning. Improper or delayed diagnosis and inadequate treatment increase the risk of serious complications, including limb amputations. Malignancies can develop secondary to chronic leg ulcers. About 2.4% of ulcers arising from chronic venous stasis undergo malignant transformation. Squamous cell carcinoma is the most common type of malignancy found in chronic leg ulceration biopsies. Basal cell carcinoma, sarcoma, and melanoma have all been documented infrequently. In the case described here, we found lymphoma of the marginal zone of mucosa-associated lymphoid tissue (MALT), which is an extremely rare cutaneous neoplasm of the lower extremities, but one that may have an association with autoimmune diseases.

Resumo Úlceras crônicas dos membros inferiores são uma condição bastante comum entre adultos, na maioria das vezes causadas por insuficiência venosa crônica. Independente da sua causa principal, úlceras crônicas dos membros estão geralmente associadas a sintomas significativos, prejudicando o funcionamento diário. O diagnóstico inadequado ou tardio e o tratamento inadequado aumentam o risco de complicações graves, inclusive o risco de amputação do membro. Malignidades podem se desenvolver secundariamente em úlceras crônicas da perna. Em torno de 2,4% das úlceras decorrentes de estase venosa crônica sofrem transformação maligna. O carcinoma de células escomosas é o tipo de malignidade mais comum que pode ser encontrada em biópsias de úlceras crônicas da perna. Carcinoma basocelular, sarcoma e melanoma foram raramente documentados. No caso aqui descrito, encontramos linfoma da zona marginal do tecido linfoide associado à mucosa (mucosa-associated lymphoid tissue, MALT), o qual é uma neoplasia cutânea dos membros inferiores extremamente rara mas que pode estar associada a doenças autoimunes.

Pre-malignant signs of gastric MALT lymphoma

Mucosa-associated lymphoid tissue (MALT) lymphoma is the most common type of extra-nodal non-Hodgkin lymphoma, which mostly involves the stomach. The clinical suspicion and diagnosis are often challenging because of the lack of specific symptoms and conventional endoscopic findings. Three magnifying endoscopic signs of the gastric mucosa have been described as highly specific to the diagnosis of MALT lymphoma, such as (i) tree-like appearance of the microvessels; (ii) non-structural area; and (iii) ballooning crypt pattern. We report the case of a middle-aged woman in which these signs appeared chronologically over a period of 2 years, showing the association of the sequence of the endoscopic findings and the final histological diagnosis of gastric MALT lymphoma.

Nuevos retos en el tratamiento de la infección por helicobacter pylori / New challenges in the treatment of the infection for helicobacter pylori

RESUMEN La infección por helicobacter pylori afecta aproximadamente al 50% de la población mundial, es causante de gastritis crónica, úlcera péptica, cáncer gástrico y linfoma del tejido linfoide asociado a la mucosa. Desde su descubrimiento, la erradicación ha sido uno de los más importantes retos en Gastroenterología. En muchos países se desconoce la prevalencia de resistencia primaria del microorganismo a los diferentes antibióticos que empíricamente se utilizan, y por no realizar pruebas de rutina que verifican su erradicación en la práctica diaria, se ignora la efectividad de los esquemas prescritos. El incremento progresivo de la resistencia a la claritromicina y metronidazol, unido a una ausencia de antibioticoterapia alternativa, desafía la capacidad para eliminar de manera efectiva a ésta bacteria. El subcitrato de bismuto ha resurgido y su adición en la terapia ha permitido aumentar las tasas de curación por encima del 90%. Actualmente se invoca que para mejorar la eficacia en el tratamiento se debe combinar una supresión potente del ácido gástrico en tratamientos combinados cuádruples con una duración de 14 días, para la mayoría de los casos. La adherencia al tratamiento es crucial para obtener buenos resultados terapéuticos.

ABSTRACT The infection for helicobacter pylori affects approximately to the world population's 50%, it is causing of chronic gastritis, peptic ulcer, gastric cancer and linfoma associated to the mucous one. From their discovery, the eradication has been one of the most important challenges in Gastroenterología. In many countries the prevalencia of primary resistance is ignored from the microorganism to the different antibiotics that empirically they are used, and for not carrying out routine tests that verify its eradication in the daily practice, the effectiveness of the prescribed outlines it is ignored. The progressive increment of the resistance to the claritromicina and metronidazol, together to an absence of alternative antibioticotherapy, challenges the capacity to eliminate from an effective way to this bacteria. The bismuth subcitrato has resurged and its addition in the therapy has allowed to increase the cure rates above 90%. At the moment it is invoked that to improve the effectiveness in the treatment, that is should combine a potent suppression of the gastric acid in combined quadruple treatments with a duration of 14 days, for most of the cases. The adherence to the treatment is crucial to obtain therapeutic good results.

Adenocarcinoma pulmonar sincrónico y linfoma primario de tejido linfoide asociado a mucosa pulmonar / Synchronous pulmonary adenocarcinoma and primary lymphoma of lung mucosa-associated lymphoid tissue

Los linfomas derivados del tejido linfoide asociado a las mucosas (MALT) son entidades poco frecuentes, de bajo grado de malignidad con escaso o nulo compromiso ganglionar y representan cerca del 80% de los linfomas primarios pulmonares. La aparición sincrónica con adenocarcinoma de pulmón es un hallazgo extremadamente infrecuente. Presentamos el caso de un hombre de 68 años, ex-tabaquista, en quien durante el seguimiento de un nódulo pulmonar se identificó un segundo nódulo y la biopsia quirúrgica confirmó el diagnóstico de ambas neoplasias.

The lymphomas of mucosa-associated lymphoid tissue (MALT), are uncommon entities, of low grade of malignancy with very infrequent or no lymph node involvement. They represent about 80% of the primary pulmonary lymphomas. The synchronous appearance with lung adenocarcinoma is an extremely rare finding. We present the case of an ex-smoker 68-year-old man, in whom, in the follow-up of a pulmonary nodule, a second pulmonary nodule was found. The surgical biopsy confirmed the diagnosis of both neoplasms.

Primary bilateral and symmetric MALT lymphoma of the lacrimal sac mimicking chronic dacryocystitis / Linfoma MALT primário, bilateral e simétrico do saco lacrimal simulando dacriocistite crônica

Abstract We report a case of primary bilateral mucosa associated lymphoid tissue (MALT) lymphoma of the lacrimal sac. MALT lymphoma is a subtype of Non-Hodgkin's Lymphoma of the ocular adnexa. When the primary site of the lymphoma is the lacrimal sac, it mimics chronic dacryocystitis. This may delay diagnosis, with potentially lethal results.

Resumo Descrevemos um caso de um linfoma MALT bilateral, simétrico e primário de saco lacrimal. O linfoma MALT é um subtipo do Linfoma Não-Hodkin dos anexos oculares. Quando o local primário do linfoma é o saco lacrimal, ele pode simular uma dacriocistite crônica. Essa situação pode atrasar o diagnóstico e ter consequências fatais.

Linfoma primario de pulmón tipo malt: presentación de caso clínico / Primary lung lymphoma MALT Type: Clinical case presentation

Antecedentes:Background: Los Linfomas Pulmonares constituyen menos del 15 % de todos los linfomas. Los linfomas primarios en pulmón son una entidad de poca frecuencia, más aún el subtipo llamado MALT (linfoma de tejido linfoide asociado a mucosas) estos representan apenas al 1 % de todos los linfomas y 0,5 % de todos los tumores pulmonares. Se caracterizan por proli-feración clonal linfoide que afecta a los bronquios o al parénquima pulmonar de forma unilateral o bilateral sin que exista evidencia de afectación extra torácica al momento de su diagnóstico. Caso Clinico: paciente de 33 años atendido en servicio de Neumología del Instituto Nacional Cardiopulmonar de Tegucigalpa, Honduras por historia de dolor pleurítico en hemitórax derecho, tos con expecto-ración hemoptoica de 3 semanas de evolución acompañado de estertores crepitantes en hemitórax anterior derecho con diagnóstico histológico e inmunohistoquimico mediante biopsia de linfoma pulmonar de tipo Malt. Discusión: Es importante tener en cuenta a este tipo de tumores, dado que los síntomas, signos clínicos y los estudios de imágenes son inespecíficos. (5) teniendo en cuenta los diagnósticos diferenciales de causas no infecciosas, como la enfermedad metastásica, la granulomatosis de Wegener, los linfomas, las leucemias, las neumonías criptogénicas, la enfermedad tromboembólica y las enfermedades del colágeno...(AU)

Síndromes linfoproliferativos gastrointestinales / Gastrointestinal lymphoproliferative syndrome

The gastrointestinal lymphoid system plays a relevant role. The daily and continuous interaction between gastrointestinal lymphocytes with food and intestinal microbes requires precise functioning. The pathologic spectrum of lymphocyte malfunction results in lymphomas. MALT lymphoma is the most frequently diagnosed lymphoma, but there are other lymphoproliferative diseases such as diffuse large B cell lymphoma, mantle cell lymphoma and T associated lymphoma. The gastroenterologist and the endoscopist need to know these diseases in detail to achieve early diagnosis and treatment.

El sistema linfoide de defensa abdominal tiene un relevante rol en el buen funcionamiento sistémico. La interacción diaria y continua con patógenos alimentarios y microbios comensales intestinales precisa un estrecho funcionamiento. Las alteraciones linfoides clonales favorecen el desarrollo de linfomas de diversos tipos. Si bien, el linfoma asociado a tejido linfoide de mucosas (MALT) es el más conocido en contexto de su asociación con Helicobacter pylori, el tracto gastrointestinal se puede ver afectado por otros linfomas como el linfoma difuso de células grandes B y linfomas indolentes como el linfoma folicular, el linfoma del manto y el linfoma T asociado a enteropatía. El gastroenterólogo y endoscopista precisan conocer en detalle estas entidades para un oportuno diagnóstico y adecuado tratamiento.

Splenic marginal zone lymphoma: a literature review of diagnostic and therapeutic challenges

ABSTRACT Splenic marginal zone lymphoma (SMZL) is a low-grade B-cell non-Hodgkin's lymphoma characterized by massive splenomegaly, moderate lymphocytosis with or without villous lymphocytes, rare involvement of peripheral lymph nodes and indolent clinical course. As a rare disease, with no randomized prospective trials, there is no standard of care for SMZL so far. Splenectomy has been done for many years as an attempt to control disease, but nowadays it has not been encouraged as first line because of new advances in therapy as rituximab, that are as effective with minimal toxicity. Facing these controversies, this review highlights advances in the literature regarding diagnosis, prognostic factors, treatment indications and therapeutic options.

Oral Nodular Lesions in Patients with Sjögren's Syndrome: Unusual Oral Implications of a Systemic Disorder

Abstract Sjögren's syndrome (SS) is a systemic chronic autoimmune disorder affecting the lacrimal and salivary glands. SS may manifest as primary SS (pSS) or secondary SS (sSS), the latter occurring in the context of another autoimmune disorder. In both cases, the dry eyes and mouth affect the patient's quality of life. Late complications may include blindness, dental tissue destruction, oral candidiasis and lymphoma. This paper reports two cases of SS, each of them presenting unusual oral nodular lesion diagnosed as relapsed MALT lymphoma and mucocele. The importance of the diagnosis, treatment and management of the oral lesions by a dentist during the care of SS patients is emphasized, as the oral manifestations of SS may compromise the patient's quality of life.

Resumo A síndrome de Sjögren (SS) é uma doença autoimune crônica sistêmica que afeta as glândulas lacrimal e salivar. A SS pode se manifestar como SS primária (SSp) ou SS secundária (SSs), a última ocorrendo em conjunto com outra desordem autoimune. Em ambos os casos, os olhos secos e a boca seca afetam a qualidade de vida do paciente. As complicações tardias podem incluir cegueira, destruição dos tecidos dentários, candidíase oral e linfoma. Este artigo relata dois casos de SS, cada um apresentando lesão nodular oral incomum diagnosticada como linfoma MALT reincidente e mucocele. A importância do diagnóstico, tratamento e manejo das lesões orais por um cirurgião-dentista durante o atendimento de pacientes com SS é enfatizada, pois as manifestações orais da SS podem comprometer a qualidade de vida do paciente.

Efficacy of Positron Emission Tomography/Computed Tomography in Gastric Mucosa-associated Lymphoid Tissue Lymphoma / 대한소화기학회지

BACKGROUND/AIMS: This study evaluated the diagnostic efficacy of fluorine-18 fluorodeoxyglucose PET/CT (F-18 FDG PET/CT) for patients with gastric mucosa-associated lymphoid tissue (MALT) lymphoma and examined the association between FDG avidity and the clinical factors affecting lesions. METHODS: Among the patients diagnosed with gastric MALT lymphoma, 16 who underwent a PET/CT for gastric MALT lymphoma were semi-quantitatively and qualitatively tested for FDG avidity of lesions in the stomach. Retrospectively collected data was analyzed to investigate the clinicoradiological factors and endoscopic findings between the patients with positive F-18 FDG PET/CT scans and those with negative scans. RESULTS: Eight of the 16 patients showed FDG avidity. When comparing the size of lesions in the stomach, the patients with FDG avidity had significantly larger lesions than those without (28.8 mm vs. 15.0 mm, p=0.03). The FDG-avid group has a significantly higher rate of positive CT scans than the non-avid group (75% vs. 13%, p=0.03). According to the endoscopic finding of the lesions, FDG avidity was pronounced with 75% of the protruding tumors, and 100% of the erosive-ulcerative types, which are a type of depressed tumors. CONCLUSIONS: When gastric MALT lymphoma is large, when lesions are found using abdominal CT scans, and the macroscopic appearance of a lesion is that of a protruding tumor or erosive-ulcerative type of depressed tumor, there is a high probability that such patients may have a positive F-18 FDG PET/CT scan.

Clinicopathologic Study of Chromosomal Aberrations in Ocular Adnexal Lymphomas of Korean Patients

PURPOSE: The incidence and clinical correlation of MALT1 translocation and chromosomal numerical aberrations in Korean patients with ocular adnexal mucosa associated lymphoid tissue (MALT) lymphoma have not yet been reported. We investigated the incidence and clinicopathologic relationship of these chromosomal aberrations in ocular adnexal MALT lymphomas in a Korean population. METHODS: Thirty ocular adnexal MALT lymphomas were investigated for the t(11;18) API2-MALT1, t(14;18) IgH-MALT1 translocations and chromosomes 3 and 18 aneuploidies using fluorescence in situ hybridization. Patient medical records were reviewed retrospectively for information on demographics and clinical characteristics, including treatment response. RESULTS: The MALT1 gene rearrangement was found in one out of 30 cases. The t(14;18) IgH-MALT1 translocation was demonstrated in only one case (3.3%), and the t(11;18) API2-MALT1 translocation was not found in any of the cases. Trisomy 3 was observed in three ocular adnexal MALT lymphomas (10.0%), and five cases showed trisomy 18 (16.7%). Translocation positive cases also showed trisomy 18. One case of tumor relapse showed trisomy 18 only in the recurrent biopsies. There were no statistically significant correlations between chromosomal aberrations and clinical characteristics and treatment responses. CONCLUSIONS: Translocations involving the MALT1 gene are not common in Korean ocular adnexal MALT lymphomas. The t(14;18) translocation was detected in only one out of 30 cases, and the t(11;18) translocation was not found at all. Furthermore, the chromosomal aberrations found in this study had no prognostic implications.

The Pleural Sandwich Sign in Two Cases of Primary Pleural Lymphoma

The sandwich sign is used to describe mesenteric lymphoma in which mesenteric vessels and fat are enveloped by enlarged mesenteric lymph nodes. We present two cases of primary pleural lymphoma demonstrating the "pleural sandwich sign". Contrast-enhanced computed tomography showed conglomerated parietal pleural and extrapleural masses encasing the intercostal arteries. Histopathological examinations confirmed low grade marginal zone B-cell lymphoma in an 80-year-old man and diffuse large B-cell lymphoma in a 68-year-old man. The pleural sandwich sign may suggest the diagnosis of primary pleural lymphoma.

Linfoma tipo Malt pulmonar: presentación de un caso y revisión de la bibliografía / Pulmonary Malt lymphoma: case report and literature review

El linfoma pulmonar primario es de presentación poco frecuente y representa solo el 0,5 a 1 por ciento de los procesos tumorales malignos del pulmón. Se presenta el caso de un paciente con diagnóstico histológico de linfoma tipo MALT de pulmón, ingresado y operado en el Hospital Saturnino Lora Torres de Santiago de Cuba. El paciente fue seguido por consulta de cirugía y oncología con más de 3 años de intervalo libre de enfermedad. El linfoma tipo Malt es una entidad poco frecuente, con diagnóstico preoperatorio prácticamente nulo, lo que dificulta su manejo adecuado(AU)

Primary pulmonary lymphoma is very uncommon, representing only 0.5 - 1 percent of malignant lung tumoral malignancies. Here is a patient with histologically confirmed lung MALT lymphoma, who was operated on at Saturnino Lora Torres hospital in Santiago de Cuba province. The patient was followed up by the surgery and oncology service and he was disease-free for over three years. Malt-type lymphoma is rare, its diagnosis before surgery is practically non-existent, which hinders proper management of a case(AU)

Metachronous early gastric adenocarcinoma presenting coinstantaneously with complete remission of stage IV gastric MALT lymphoma

Adenocarcinoma and lymphoma represent the two most common malignant tumours of the stomach, with both neoplasms being associated with infection by Helicobacter pylori. However, the presence of lymphoma and adenocarcinoma in the same patient is a rare entity with synchronous neoplasms being more common than metachronous types. We report a case of stage IV gastric MALT lymphoma of the gastric angle with infiltration of the bone marrow successfully treated with chemotherapy and the occurrence of metachronous early gastric adenocarcinoma of the fundus presenting 1 year after the diagnosis of the lymphoma

A Case of Ileal Mucosa-associated Lymphoid Tissue Lymphoma Accompanied by Luminal Stricture and Arterial Spurting / 대한소화기학회지

Primary small intestinal lymphoma is relatively uncommon. Small bowel tumors are difficult to diagnose, because they are usually asymptomatic in the initial phase, and they are not easily detected by traditional methods of investigating the small intestine. This case shows a successfully detected and treated gastrointestinal bleeding from rare ileal mucosa-associated lymphoid tissue lymphoma, using double balloon endoscopy.

Primary Mucosa-associated Lymphoid Tissue Lymphoma of the Esophagus, Manifesting as a Submucosal Tumor / 대한소화기학회지

We report a case of primary mucosa-associated lymphoid tissue (MALT) lymphoma in the esophagus that manifested as a large submucosal tumor (SMT). Primary esophageal lymphoma is very rare, occurring in less than 1% of all patients with gastrointestinal lymphoma. Only a few cases of MALT lymphoma in the esophagus have been reported in the English literature. A 53-year-old man was referred to Dongguk University Ilsan Hospital (Goyang, Korea) in July 2012 for further evaluation and treatment of an esophageal SMT. Endoscopy showed a cylindrically elongated submucosal mass with normal overlying mucosa in the mid esophagus, 25-30 cm from the incisor teeth. He underwent surgery to confirm the diagnosis. Pathologic findings showed diffuse small atypical lymphoid cells which were stained with Bcl-2, CD20, but not with CD3, CD5, CD23, Bcl-6, or cyclin D1. These cells showed a positive monoclonal band for immunoglobulin heavy chain gene rearrangement. Based on the pathological, immunohistochemical, and molecular biological features, the esophageal mass was diagnosed as extranodal marginal zone B-cell lymphoma of the MALT type.

Primary Malt Lymphoma of the Common Bile Duct

Primary mucosa-associated lymphoid tissue (MALT) lymphoma arising in the common bile duct (CBD) is extremely rare. In our case of MALT lymphoma, CT and MRI showed long, segmental, irregular wall thickening of the CBD and minimal dilatation of the upstream bile duct. A preoperative diagnosis of cholangiocarcinoma was made, but histologic evaluation confirmed MALT lymphoma of the CBD. We herein present a rare case of MALT lymphoma of the CBD with CT and MRI findings.

Primary cutaneous marginal zone lymphoma (immunocytoma like) with lymphoepithelioid or Lennert's lymphoma like involvement of nodes.

Primary cutaneous marginal zone lymphomas (PCMZL) have a wide range of morphology from tumors with monocytoid B cells to those composed entirely of plasma cells and the T-cell rich variants. We report a 60-year-old male with a PCMZL rich in plasma cells of the foot with a lymphoepithelioid-like pattern of dissemination to the lymph nodes posing problems in the diagnosis. The patient had a lesion on the dorsum of the foot which histologically revealed dense perivascular collections of lymphoid cells and plasma cells amidst fibrous tissue. Though the plasma cells did show light chain restriction, CD20 and CD3 did not reveal an overwhelming B/T-cell population and hence a diagnosis of a reactive process was offered. Subsequently the patient developed inguinal nodes with diffuse loss of architecture and replacement by epithelioid histiocytes and reactive T cells with few large B cells (lymphoepithelioid-like pattern). On pathology review it was realized that the two lesions may be related and clonality studies were asked for. The skin lesion showed clonally rearranged IgH receptor while the T-cell receptor rearrangement was negative. The patient developed disseminated disease and received six cycles of chemotherapy with partial response and 6 years after the initial presentation was alive with nonprogressive disease. Thus, the polymorphous background in PCMZL is evolving and an immunocytoma-like tumor can show a T-cell rich or Lennert's like growth pattern of spread and early recognition these odd patterns may aid in appropriate management of patients.