The Current Status and Research Progress of Antiviral Therapy in HCV-Associated Lymphoma --Review / 中国实验血液学杂志

Hepatitis C virus (HCV) is one of the leading causes of chronic liver disease. HCV is not only related to hepatic malignancies but may also promote lymphoid neoplasms. Currently, research has confirmed HCV-related lymphoma, including marginal zone lymphoma (MZL), lymphoplasmacytic lymphoma (LPL), follicular lymphoma (FL), diffuse large B-cell lymphoma (DLBCL), and Burkitt lymphoma (BL). Many types of research have shown that antiviral therapy can improve or even remission several HCV-related lymphomas. The direct-acting antiviral agents (DAAs) (such as NS5A protease inhibitors, NS4/4A protease inhibitors and viral polymerase inhibitors) have shown clinical advantages of high efficacy and low side effects for both virus elimination and tumor regression in several HCV-related lymphomas, which may make the selected HCV-related lymphoma patients treated without chemotherapy. In this review the research progress and development direction of antiviral therapy in treating HCV-related lymphoma has summarized briefly.

MALT lymphomas - Treated with chemotherapy or radiotherapy: clinical features, prognostic factors and survival

MALT lymphomas are a group of extranodal indolent lymphomas that usually present as stage IE. To clarify clinical features, treatment alternatives and outcomes, we evaluated 38 patients treated with chemotherapy or radiotherapy between 2000 and 2011. MALT lymphoma patients identified according to WHO classification and treated at KCCC between 2000 and 2011 were included in this study. Demographic and clinical data are presented as means or medians. Overall survival was estimated using the Kaplan-Meier method. Survival rates were compared using the log-rank test. A p value < 0.05 was considered significant. The median age of the patients was 49 years and the male to female ratio was 2:1. Gastric MALT accounted for 63% of all patients and the most common presenting symptom was abdomen pain and dyspepsia. The common extra gastric sites were salivary glands, lung and orbit. 90% of the patients presented with early stage disease. Two patients had history of pre-existing autoimmune disease. Even among patients who had failed prior antibiotic therapy for Helicobacter pylori, treatment with chemotherapy achieved good results with 5 year survival of 80%. MALT lymphomas are indolent neoplasm's with excellent long term outcome. There is no significant difference in survival between gastric and extra-gastric MALT lymphoma

Linfoma esplénico de zona marginal como causa de esplenomegalia masiva: reporte de caso clínico y revisión de la literatura / Splenic marginal zone lymphoma as cause for massive splenomegaly: a clinical case and literature review

Massive splenomegaly is in the which the growth of the spleen has spread to other quadrants of the abdomen. It is produced by a limited number of pathologies, both benign and malignant. It is presented a case of a 62 year-old woman who is consulting for four years of progressive increase in her abdominal volume, associated to the feeling of abdominal fullness, dyspnea on moderate exertion and lower extremities edema. At the physical examination was observed massive splenomegaly and jaundice. The hemogram showed pancytopenia and a lymphocyte count of80 percent. The myelogram revealed marrow infiltration by lymphocytes of mature appearance. Flow cytometry of peripheral blood showed 70 percent of lymphocytes, which expressed B cells markers CD19, CD20, CD23and FMC7 in addition to Kappa light chain restriction, suggesting marginal splenic zone lymphoma. The bone marrow biopsy showed lymphoid small cells infiltrate with positive markers CD20, CD5,CD23 and negative cyclin D1 study. BCL-2 was also positive. It was considered unfit to receive chemotherapy and was treated with 4 cycles of rituximab, with significant decrease of splenic size.

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.

Mucosa-associated lymphoid tissue lymphoma of the trachea: case report / Linfoma do tecido linfoide associado à mucosa de traqueia: relato de caso

CONTEXT: Mucosa-associated lymphoid tissue (MALT) lymphomas are most commonly found in the stomach, lungs, orbital soft tissue, salivary glands and thyroid. Involvement of the trachea is extremely rare. CASE REPORT: This report describes a rare case of MALT lymphoma of the trachea in a 71-year-old woman who presented with a one-year history of coughing, dyspnea, hoarseness and weight loss. There was an infiltrative lesion in the mid-trachea. The anatomopathological diagnosis was only made from the fifth endoscopic biopsy attempt. Immunochemotherapy consisting of rituximab, cyclophosphamide, vincristine and prednisone (R-COP) induced complete remission of the symptoms and endoscopic lesion. CONCLUSIONS: MALT lymphoma of the trachea is extremely rare and indolent disease. It has to be considered in the differential diagnosis of airway lesions. It is crucial to obtain an anatomopathological diagnosis from a specialized pathologist. Immunochemotherapy with R-COP induced complete remission of the disease.

CONTEXTO: Linfomas de tecido linfoide associado à mucosa (TLAM) são mais comumente encontrados no estômago, pulmões, órbita, glandulas salivares e tireoide. O envolvimento da traqueia é extremamente raro. RELATO DE CASO: Relato de caso raro de linfoma TLAM de traqueia, em mulher de 71 anos com tosse, dispneia, rouquidão e emagrecimento há um ano. Detectou-se lesão infiltrativa na porção média da traquéia. O diagnóstico anatomopatológico só foi obtido na biópsia do quinto procedimento endoscópico. A imunoquimioterapia R-COP (rituximabe, ciclofosfamida, vincristina e prednisona) induziu remissão completa dos sintomas e da lesão endoscópica. CONCLUSÕES: Linfoma TLAM de traqueia é uma doença indolente e extremamente rara, que deve ser considerada no diagnóstico diferencial das lesões de vias aéreas. O diagnóstico anatomopatológico por um patologista experimentado é fundamental. Imunoquimioterapia com R-COP levou à remissão completa da doença.

Extranodal marginal zone B cell lymphoma of mucosa-associated lymphoid tissue type of the ocular adnexa: Retrospective single institution review of 95 patients.

Context: There are few reports on the management of extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue type (MALT), which are based on the experience of a single institution, as opposed to large multicenter randomized trials. Aim: The aim of this study was to report on the clinical experience of a single institution. Materials and Methods: A retrospective review was undertaken of records of 95 patients with MALT lymphoma of the ocular adnexa. Histologic diagnosis of MALT lymphoma was made according to established criteria, and clinical staging was carried out to determine treatment modalities. All patients were treated by external beam irradiation (30.6–45.0 Gy) after biopsy. Additional chemotherapy was performed in accordance with the clinical stage of the disease. All the patients were treated by the same hemato-oncologist and radio-oncologist. Results: Almost all patients showed complete response, except for four patients who showed partial response. In two of 95 patients, contralateral eye showed recurrence, and they were salvaged by additional radiotherapy. The 3-year overall survival and event-free survival rates were 100 and 97%, respectively, by Kaplan–Meier survival analysis. Moreover, there were no serious radiation-associated complications. Conclusions: Radiotherapy alone can be an important treatment modality for the local control and survival in patients with localized MALT lymphoma of ocular adnexa. Systemic chemotherapy should be considered in patients with advanced stage disease.

Linfoma MALT gástrico, gastritis linfocitaria y enfermedad celíaca: caso clínico con seguimiento a largo plazo, post erradicación de Helicobacter pylori y regresión del MALT. Correlación Clínico-Endoscópica-Patológica / Gastric MALT lymphoma, lymphocytic gastritis and celiac disease: case report with long-term follow-up post helicobacter pylori eradication and regression of MALT: clinical-pathological and endoscopic correlation

A case report of 61 year old female patient with diarrhea syndrome, whose study showed the presence of low-grade MALT gastric lymphoma associated with lymphocytic gastritis and celiac disease. During a six-year period of follow-up, the eradication of Helicobacter pylori determined the remission of the gastric lymphoma and lymphocytic gastritis; celiac disease showed variable activity according to their adherence to dietary treatment. We discuss possible associations and etiology of MALT lymphoma and lymphocytic gastritis, and of the latter with celiac disease.

Se presenta caso de paciente mujer de 61 años con cuadro de síndrome diarreico, cuyo estudio demuestra la presencia de linfoma MALT de bajo grado asociado a gastritis linfocitaria y enfermedad celíaca. Durante su seguimiento de 6 años, la erradicación de Helicobacter pylori, determinó la remisión del linfoma y gastritis linfocitaria, mientras la enfermedad celíaca mostró actividad variable de acuerdo con su adherencia al tratamiento dietético. Se discuten las posibles asociaciones y etiología de linfoma MALT y gastritis linfocitaria, y de esta última con enfermedad celíaca.

Pulmonary BALT Lymphoma Successfully Treated with Eight Cycles Weekly Rituximab: Report of First Case and F-18 FDG PET/CT Images

Extra marginal-zone lymphomas of the lung is a very rare tumor and it originates from bronchial-associated lymphoid tissue. A 68-yr-old woman presented with productive cough and dyspnea. A thorax computed tomography scan showed a 9 x 10 cm in size mass in the left lung and pleural effusion in the lower lobe of left lung. Positron emission tomography/computed tomography (PET/CT) revealed intense uptake foci at the upper and middle sites of left lung and slight uptake foci at the mediastinal lymph nodes which showed malignant involvement. After bronchoscopic biopsy, the diagnosis of pulmonary bronchial-associated lymphoid tissue (BALT) lymphoma was confirmed. At the end of the eight cycles weekly rituximab treatment, complete response was obtained by PET/CT findings. It is concluded that extended rituximab schedule is more effective and it would be beneficial to investigate the use of PET/CT in the diagnosis and evaluating of the treatment response of pulmonary BALT lymphoma.

Rituximab monotherapy for splenic marginal zone lymphoma with villous lymphocytes: report on long-term disease control for two patients with recurrence after splenectomy / Monoterapia com rituximab no linfoma da zona marginal esplênico com linfócitos vilosos: relato de dois casos de pacientes com controle prolongado da doença depois de recidiva após esplenectomia

CONTEXT: Splenic marginal zone lymphoma (SMZL) is a lymphoproliferative B-cell disorder that has a favorable prognosis, with estimated overall five-year survival of 70 percent. The majority of symptomatic patients undergo splenectomy, while a few receive first-line chemotherapy, especially with purine analogues. There are no specific treatment guidelines for patients for whom splenectomy fails to provide a cure. It is still unclear whether these patients should undergo cytotoxic chemotherapy, considering they have now a relapsed lymphoma (which is theoretically more aggressive), or whether they should be spared from treatments of greater toxicity, given that their disease usually develops with a more indolent course, even when it recurs. CASE REPORT: Here, we present two patients whose disease recurred after splenectomy and for whom rituximab monotherapy provided satisfactory treatment. From these cases, it can be suggested that postponement of cytotoxic treatments may be possible in at least some situations. It needs to be emphasized that the evidence to support this approach is based only on case reports, since there are no randomized clinical trials on this subject.

CONTEXTO: Os linfomas da zona marginal esplênicos constituem uma desordem linfoproliferativa de células B que apresenta um prognóstico favorável, com sobrevida global de cinco anos estimada em 70 por cento. A maioria dos pacientes sintomáticos é submetida a esplenectomia enquanto alguns recebem quimioterapia terapêutica de primeira linha, especialmente com análogos de purinas. Não existem diretrizes específicas para o tratamento dos pacientes que falham à esplenectomia: ainda é incerto se deveriam ser tratados com quimioterapia citotóxica, em virtude de apresentarem um linfoma recidivado (e teoricamente mais agressivo) ou se deveriam ser poupados de um tratamento mais tóxico pelo fato de apresentarem uma doença que usualmente se desenvolve de forma mais indolente, mesmo quando recidivada. RELATO DE CASO: Nesta publicação, são apresentados dois casos nos quais a doença recidivou após esplenectomia e que foram satisfatoriamente tratados com monoterapia com rituximabe. A observação desses casos sugere que a postergação de tratamentos citotóxicos pode ser possível pelo menos em algumas situações. Cabe ressaltar que a evidência para essa conduta é embasada apenas em relatos de caso, uma vez que não existem ensaios clínicos randomizados a respeito desse tema.

Extranodal Marginal Zone Lymphoma Occurring along the Trachea and Central Airway

Extranodal marginal zone lymphoma is a low-grade B cell lymphoma that presents with an indolent clinicopathologic nature. Although this tumor can occur in various sites, including the gastrointestinal tract and lungs, it develops and spreads extremely rarely along the trachea and central airway. We report a case of extranodal lymphoma of mucosa-associated lymphoid tissue with tracheobronchial involvement. An 83-year-old woman presented with a cough and dyspnea. Bronchoscopic evaluation confirmed diffuse, multiple nodular lesions in both the trachea and large bronchi, and she was diagnosed with an extranodal marginal zone lymphoma of the tracheobronchial tree. After systemic chemotherapy, she survived for more than 18 months.

Rituximab-CHOP Induced Interstitial Pneumonitis in Patients with Disseminated Extranodal Marginal Zone B Cell Lymphoma

A 69-year-old male was diagnosed in February 2004 with stage IV extranodal marginal zone B cell lymphoma involving the mediastinal nodes, lung parenchyma and bone marrow with high LDH. Shortness of breath developed following the 5th course of Rituximab-CHOP chemotherapy (cyclophosphamide, Vincristine, Doxorubicin, Prednisolone). Bronchoscopy guided transbronchial lung biopsy revealed interstitial thickening and type II pneumocyte activation, compatible with interstitial pneumonitis. After treatment with prednisolone a complete resolution of the dyspnea was observed. The patient was well on routine follow-up at the outpatient clinic, with no progression of lymphoma or interstitial pneumonitis.

The Clinical Characteristics and Treatment Results of Ocular Adnexal Lymphoma

PURPOSE: To assess the clinical pattern, the histopathological findings, the response to treatments, the recurrence pattern and the prognosis of malignant lymphoma in the ocular adnexa. METHODS: This study was performed on 22 total eyes from 17 patients who were diagnosed with ocular adnexal malignant lymphoma. We retrospectively analyzed the medical records for patient information including the histological classification based on age, the gender of each patient, the symptoms and signs at the initial diagnosis, the presence of binocular invasion, the findings of the surgical biopsy, the clinical stage of each patient's tumor, and the treatment methods used and their effectiveness. The mean follow-up period was 24.8 months. RESULTS: The mean age of patients studied was 46.8 years old. Six females and 11 males were included in the study. Fifteen cases consisting of 20 total eyes represented extranodal marginal zone B cell lymphoma of mucosa-associated lymphoid tissue (MALT). Five of seven patients (71.4%) whose lymphoma occurred within the conjunctiva relapsed after irradiation or chemotherapy, and four of the relapsed patients were salvaged with further therapy. CONCLUSIONS: Extranodal marginal zone B cell lymphoma of mucosa-associated lymphoid tissue (MALT) constituted 88.2% of all lymphomas involving the ocular adnexa. Lymphoma in the ocular adnexa responded well to conventional treatment, but the recurrence rate of lymphoma in the conjunctiva was significantly high.

Linfoma de tecido linfático associado a mucosa MALT, primário de fígado, com infiltraçäo de vesícula biliar e medula óssea / Mucosa associated lymphatic tissue lymphoma, MALT, primarily from the liver, with infiltration of gall bladder and bone marrow

Linfomas hepáticos primários do fígado säo raros. Linfomas indolentes de expressäo B do tipo tecido linfático associado a mucosa säo ainda mais raros e inusitados, estando descritos na literatura somente quatro casos. Os autores apresentam neste relato um caso de paciente do sexo feminino, com 73 anos de idade com queixas dolorosas e sintomatologia de comprometimento biliar. O diagnóstico de linfoma do tecido linfático associado a mucosa (MALT) foi realizado acidentalmente pelo encontro de anormalidades hepáticas no momento do ato cirúrgico. O aspecto hepático era micronodular com a presença de lesäo ulcerada, que biopsiadas revelaram o diagnóstico. O estadiamento da moléstia comprovou infiltraçäo da medula óssea pelo linfoma, dado este infrequente, e a endoscopia digestiva alta foi normal, näo revelando comprometimento deste aparelho, dado comum e mais freqüente deste tipo de linfoma.