MALT Lymphomas.

2003

**Editors Note: While this study dates back to 2003, it is still worth the reading for the information contained.**

Malek SN, Hatfield AJ, Flinn IW.

Source

The Sidney Kimmel Comprehensive Cancer Center at Johns Hopkins, 1650 Orleans Street, CRB Room 388, Baltimore, MD 21231, USA.

Abstract

Mucosa-associated lymphoid tissue (MALT) lymphomas occur in a variety of organs, including the orbit, conjunctiva, salivary glands, skin, thyroid gland, lungs, stomach, and intestine. These tumors are often localized and of indolent clinical behavior. Diagnosis is made by pathologic evaluation of a tissue biopsy. Careful staging is mandatory and tailored to the initial presentation. Staging includes a history and physical, chemistries, computed tomography scan, and bone marrow biopsy. 

This information is supplemented with an ear, nose, and throat consultation, esophagogastro-duodenoscopy, colonoscopy, endoscopic ultrasound of the stomach, and cytogenetic/immunohistochemical analysis of the tumors. Treatment is tailored to organ involvement and stage at presentation. Eradication of Helicobacter pylori using a triple anti-H. pylori regimen approved by the US Food and Drug Administration is standard therapy for all H. pylori-positive gastric MALT lymphomas. Endoscopic ultrasound- and computed tomography-staged gastric MALT stage IE tumors will achieve a complete response with this approach in approximately 60% to 90% of patients (the more superficial the tumor, the better the response). Patients with tumors that are T4 node-positive Musshoff stage IIE1 and IIE2 or tumors with adverse cytogenetics should receive radiotherapy or surgery with or without radiotherapy. Tumors with a significant high-grade component or large cell tumors with a minor low-grade MALT component should receive CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone)-based chemotherapy. Localized MALT lymphomas of the orbit, conjunctiva, salivary glands, and thyroid gland are treated successfully with radiotherapy. Surgery as first-line therapy for gastric MALT lymphomas was replaced by attempts at organ preservation. In the past, margin-free surgical excision or tumor debulking followed by radiation therapy and chemotherapy has been highly effective for gastric MALT lymphomas. 

Therefore, surgical excision of large cell or bulky tumors of the stomach, thyroid, lung, and salivary gland, followed by adjuvant radiotherapy or chemotherapy, may still be an important consideration in selected patients. Surgery still has a role for patients with relapsed or refractory low-grade disease and life-threatening hemorrhage. Disseminated MALT lymphomas are incurable and are treated primarily with chemotherapy according to symptoms.

PubMed

Pulmonary mucosa-associated lymphoid tissue lymphoma coexisting with intratumoral tuberculosis.

Nov 2012

Inoue M, Iwaki Y, Hua J, Hagihara M.

Source

Department of Hematology, Eiju General Hospital.

Abstract

A mass in the right upper lobe of the lung was observed in a chest X-ray examination of a 66-year-old woman. Pathological examination of the lung biopsy revealed a mucosa- associated lymphoid tissue (MALT) lymphoma within the lesion. A systemic survey demonstrated no other lesions, and the patient was diagnosed as having a solitary pulmonary MALT lymphoma (Stage IE). After 9 months of careful monitoring, progressive enlargement of the lung tumor and involvement of right hilar lymph nodes were observed using positron emission tomography-computed tomography. Therefore, surgical resection of the right upper lobe and right hilar lymph nodes was performed, and coexistence of MALT lymphoma with tuberculosis was identified by pathological investigations. The association of chronic inflammation with the development of MALT lymphomas has been widely accepted. In the present case, pulmonary tuberculosis may have played a role in the pathogenesis of pulmonary MALT lymphoma.

Rinsho Ketsueki - Japanese Society of Hematology

Long-term clinical outcome of gastric MALT lymphoma after eradication of Helicobacter pylori: a multicentre cohort follow-up study of 420 patients in Japan.

Apr 2012

Nakamura S, Sugiyama T, Matsumoto T, Iijima K, Ono S, Tajika M, Tari A, Kitadai Y, Matsumoto H, Nagaya T, Kamoshida T, Watanabe N, Chiba T, Origasa H, Asaka M; JAPAN GAST Study Group.

Collaborators (23)

Source

Department of Medicine and Clinical Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan. shonaka@intmed2.med.kyushu-u.ac.jp

Abstract

OBJECTIVE:

A multicentre cohort follow-up study of a large number of patients with gastric mucosa-associated lymphoid tissue (MALT) lymphoma was conducted to elucidate the long-term outcome of the disease after Helicobacter pylori eradication.

METHODS:

420 patients with gastric low-grade MALT lymphoma who had undergone successful H pylori eradication and been followed up for at least 3 years were registered from 21 participating institutes. Responders to treatment were defined as patients whose post-treatment biopsies showed complete histological response (ChR) or probable minimal residual disease (pMRD). Treatment failure was defined as the status of progressive disease or lymphoma relapse after ChR/pMRD.

RESULTS:

323 patients (77%) responded to H pylori eradication. A logistic regression analysis showed that absence of H pylori, submucosal invasion determined by endoscopic ultrasonography and t(11;18)/API2-MALT1 were independent predictors of resistance to H pylori eradication. During the follow-up periods ranging from 3.0 to 14.6 years (mean 6.5 years, median 6.04 years), the disease relapsed in 10 of 323 responders (3.1%) while progressive disease was found in 27 of 97 non-responders (27%). Thus, 37 of 420 patients (8.8%) were regarded as treatment failures. Of these 37 patients, transformation into diffuse large B cell lymphoma occurred in nine patients. Among the non-responders and relapsed patients, 17 patients were subjected to a 'watch and wait' strategy while 90 patients underwent second-line treatments including radiotherapy (n=49), chemotherapy (n=26), surgical resection (n=6), chemoradiotherapy (n=5), antibiotic treatment (n=2), rituximab monotherapy (n=1) or endoscopic resection (n=1). Probabilities of freedom from treatment failure, overall survival and event-free survival after 10 years were 90%, 95% and 86%, respectively. Cox multivariate analysis revealed endoscopic non-superficial type to be an independent prognostic factor for adverse freedom from treatment failure, overall survival and event-free survival.

CONCLUSIONS:

The excellent long-term outcome of gastric MALT lymphoma after H pylori eradication was confirmed by this large-scale follow-up study.

GUT Int. Journal of Gastroenterology and Hepatology

Research Progress on the Etiology and Pathogenesis of MALT Lymphoma

2012 

[Article in Chinese]

Wang XC, Ke XY.

Source

Department of Hematology, Peking University Third Hospita, Beijing 100083, China.

Abstract

Mucosa-associated lymphoid tissue (MALT) lymphoma originated outside the lymph nodes is low grade malignant B celllymphoma. It is the most frequent type of marginal zone non-Hodgkin's lymphoma, that usually occurs in the stomach, salivary gland, thyroid gland and orbital adnexa. Gastric MALT lymphoma accounts for 50% of MALT lymphoma. GastricMALT lymphoma has been confirmed to relate with Helicobacter pylori (HP) infection, its main pathogenesis is immune reaction, but some patients with chromosome translocation have no response to HP eradication, suggesting presence of other unknow pathogenesis. The chromosome translocations in MALT lymphoma are t(11;18)(q21;q21), t(1;14)(p22;q32), t(14;18)(q32;q21), t(3;14)(p14.1;q32). Recent studies show some new chromosomal abnormalities such as 6q23.3/A20 and so on, which have some effects on clinical course and prognosis. MALT lymphoma with chromosome abnormalities usually activate common NF-κB molecular pathway, and persistent active NF-κB pathway drives tumor cell proliferative and active, resulting in lymphoma incidence. In this articl, the advances in the etiology and pathogenesis of MALT lymphoma were reviewed.

PubMed

The many faces of marginal zone lymphoma.

2012

Zinzani PL.

Source

1Istituto di Ematologia "Seràgnoli" Università di Bologna, Bologna, Italy.

Abstract

Indolent B-cell lymphomas that are supposed to derive from the marginal zone (marginal zone lymphomas [MZLs]) include 3 specific entities: extranodal marginal zone lymphoma (EMZL) or mucosa-associated lymphatic tissue (MALT) lymphoma, splenic MZL (SMZL), and nodal MZL (NMZL). The clinical and molecular characteristics are different for each entity, with some shared phenotypic and genetic features. EMZL is the most common entity, accounting for approximately 70% of all MZLs. These neoplasms can arise at virtually any extranodal site and are commonly associated with chronic antigenic stimulation either as a result of infection (eg, Helicobacter pylori in the stomach) or autoimmune disease (eg, Sjögren syndrome and salivary glands). Several chromosomal translocations were also identified in EMZL, accounting in the aggregate for approximately one-third of all cases. SMZL accounts for approximately 20% of all MZLs. Patients typically present with an enlarged spleen and involvement of abdominal lymph nodes and BM. Approximately 40%-50% of SMZLs are associated with deletions of chromosome 7q. NMZL is the less common entity, representing approximately 10% of all MZLs. Patients with NMZL, by definition, have lymph node-based disease without involvement of the spleen or extranodal sites. The molecular pathogenesis of NMZL is still unknown.

Hematology

Extranodal marginal zone B-cell lymphoma of the lung: experience with fludarabine and mitoxantrone-containing regimens.

Dec. 2012

Zinzani PL, Pellegrini C, Gandolfi L, Casadei B, Derenzini E, Broccoli A, Quirini F, Argnani L, Pileri S, Celli M, Fanti S,Poletti V, Stefoni V, Baccarani M.

Source

Institute of Hematology "L. e A. Seràgnoli", University of Bologna, Bologna, Italy. pierluigi.zinzani@unibo.it.

Abstract

Bronchial-associated lymphoid tissue (BALT) lymphoma is an extranodal primary pulmonary lymphoma. The optimal therapy for this rare disease is still debated, and few heterogeneous data are available in literature. The aim of our study was to critically review data of patients with BALT lymphoma treated in first-line therapy with fludarabine and mitoxantrone-containing regimens (with or without rituximab) to investigate the effectiveness and the safety of this approach and patients' survival. An observational retrospective study was performed on homogenous clinical data from 17 patients with biopsy-proven diagnosis of BALT. All the patients were treated with fludarabine and mitoxantrone-containing regimen therapy. Radiological findings were also reviewed to assess the role of (18) fluoro-deoxyglucose positron emission tomography in the initial assessment and in the monitoring of this extranodal lymphoma. A high percentage of response was observed: 82.3% of patients achieved a complete response, 11.8% a partial response. Furthermore, a very remarkable progression-free survival (71%) and overall survival (100%) were estimated at 14 years. No relevant toxicities were registered. Our results support the use of fludarabine and mitoxantrone-containing regimens as first-line therapy in the treatment of BALT lymphoma even if further data are necessary to consolidate our findings. Positron emission tomography scanning may provide additional valuable information in the assessment of BALT lymphoma

Keywords:

• extranodal marginal B-cell lymphoma;
• BALT;
• fludarabine-containing regimen;
• rituximab;
• lung lymphoma

Wiley Online