Extranodal marginal zone lymphoma of MALT type

Extranodal marginal zone lymphomas of MALT type are a rare form of indolent — slow-growing — mature B-cell lymphoma. They account for around 7–10% of B-cell non-Hodgkin lymphomas and up to 50% of lymphomas that primarily affect the stomach.

“Extranodal” means that the primary site where the lymphoma appears is outside the lymph nodes. MALT is mucosa-associated lymphoid tissue and is found in specific areas, such as the mucous membranes of the eyes, the intestine, and so on, or, for example, in Peyer’s patches, which are cluster-shaped areas of lymphatic tissue lining the inner walls of the small intestine. These are areas of the immune system where a large number of immune cells accumulate, especially lymphocytes and dendritic cells. The term “MALT” is the acronym for “mucosa-associated lymphoid tissue”. Most cases occur in adults, with an average age of 60 years, and there is a slight predominance in women.

Regarding the causes of MALT lymphoma, its development is usually preceded by a chronic inflammatory or infectious process that leads to the recruitment of B lymphocytes to this site. As a result of this persistent stimulation, the lymphocytes eventually become malignant. For this reason, these lymphomas are more common in patients with autoimmune diseases or chronic infections. 

In this respect, the most characteristic example is gastric MALT lymphoma. Infection with a bacterium that infects the human gastric epithelium, Helicobacter pylori — in 90% of cases — leads to the appearance of MALT tissue in the form of follicular gastritis. From this gastritis, genetic and molecular changes may occur that give rise to the development of MALT lymphoma.

There are other forms of MALT lymphoma that have been linked to other infections, such as:

• Cutaneous MALT lymphomas: Borrelia burgdorferi
• Ocular lymphomas: Chlamydia psitacci
• Intestinal lymphoma: Campylobacter jejuni

Autoimmune diseases such as Sjögren’s syndrome or Hashimoto’s thyroiditis are also associated with a higher risk of developing MALT lymphomas.

Regarding the particular clinical features of MALT lymphoma, these will vary depending on the location of the lymphoma. In gastric MALT lymphomas, patients usually consult their doctor with gastric symptoms such as heartburn, epigastric or abdominal pain and, in cases of more advanced lymphoma, gastrointestinal bleeding. This type of lymphoma is very slow-growing and usually remains localised, although it may eventually spread to other areas such as the lungs, intestines or bone marrow. In 10% of patients with MALT lymphoma, the disease transforms into an aggressive lymphoma with a poor prognosis.

As for diagnosis, in addition to the tests common to other non-Hodgkin lymphomas (NHL), in gastric MALT lymphomas a fibrogastroscopy must be performed with multiple biopsies from the different gastric regions. It is also important to carry out an endoscopic ultrasound study to assess the depth of lymphoma infiltration; this has been directly linked to response to treatment. Testing for Helicobacter pylori is mandatory in gastric MALT lymphoma.

In addition to the usual morphological and immunophenotypic study, cytogenetic analysis is recommended to detect the most important cytogenetic alterations:

t(11;18), which is associated with a poor response to eradication antibiotic treatment, t(14;18), t(1;14) and t(3;14).

In cases of localised gastric MALT lymphoma, which account for the majority of cases, eradication of the bacterium Helicobacter pylori — present in the vast majority of cases — with antibiotics leads to the disappearance of the lymphoma at microscopic level in most cases, although responses may be slow.

The commonly accepted initial therapeutic strategy is antibiotic treatment combined with proton pump inhibitors. In our setting, due to the increasing number of H. pylori-resistant cases, the most recommended regimens are quadruple therapies — clarithromycin, amoxicillin, metronidazole and omeprazole — for 10–14 days. Other rescue regimens are available for cases in which the bacterium is resistant to first-line treatment.

In cases of localised disease that does not resolve with antibiotics — H. pylori-negative cases or those with the t(11;18) translocation — radiotherapy may be given, with an excellent response. In cases of advanced disease, immunochemotherapy will be necessary, usually based on agents such as chlorambucil or bendamustine, combined with rituximab — a monoclonal antibody against the CD20 receptor, which is specifically present on neoplastic lymphoid cells. In frail patients who are not candidates for immunochemotherapy, rituximab may be administered as monotherapy, although with a lower response rate and shorter progression-free survival. Once achieved, remission remains stable in 90% of cases.

In cases of localised non-gastric MALT lymphoma, surgery or radiotherapy — maximum doses of 24 Gy — may be considered, for example in cutaneous or ocular adnexal lymphomas, with very good disease control.

Most MALT-type lymphomas have an indolent course and respond to local targeted therapies, such as eradication of Helicobacter pylori, surgery or radiotherapy, with a 5-year survival of 90%.

• MALT lymphoma (gastric and non-gastric). Lymphoma Action

• MALT lymphoma. Macmillan Cancer Support

• MALT Lymphoma. Cleveland Clinic

TESTIMONIAL MATERIALS

• Ebook “Here and now: Álvaro’s story”.
• Ebook “Here and now: Clàudia’s story”

You can order the booklets in paper format for free delivery in Spain by e-mail: imparables@fcarreras.es

BONE MARROW TRANSPLANT

• Bone Marrow Transplant Guide. Josep Carreras Foundation (content in Spanish)
• What is HLA and how does it work? Josep Carreras Foundation (content in Spanish)
• Graft-versus-Host Disease. Josep Carreras Foundation (content in Spanish)
• History of Bone Marrow Transplantation. Josep Carreras Foundation (content in Spanish)
• How is the search for an anonymous donor conducted? Josep Carreras Foundation (content in Spanish)

FOOD

• How to maintain a healthy diet during treatment? Josep Carreras Foundation (content in Spanish)
• Nutrition guide. Leukemia & Lymphoma Society

OTHER

• Ideas on what to take with me to the isolation chamber. Josep Carreras Leukaemia Foundation (content in Spanish)
• Travel tips for people with cancer. Josep Carreras Leukaemia Foundation (content in Spanish)
• Physiotherapy manual for haematological and transplant patients. Josep Carreras Leukaemia Foundation (content in Spanish)
• Prevention and treatment of oral mucositis. Josep Carreras Leukaemia Foundation (content in Spanish)
• Oral hygiene in oncohaematological patients. Josep Carreras Leukaemia Foundation (content in Spanish)
• Fertility manual: Suffering from blood cancer and becoming a parent. Josep Carreras Leukaemia Foundation (content in Spanish)
• Skin care in the oncohaematological patient. Josep Carreras Leukaemia Foundation (content in Spanish)
• Aesthetic Oncology Manual. Josep Carreras Leukaemia Foundation (content in Spanish)
• Leukaemia and sexuality. Josep Carreras Leukaemia Foundation (content in Spanish)
• 7 ways to wear a scarf. Josep Carreras Leukaemia Foundation (content in Spanish)

In Spain there is a large network of associations for haematological cancer patients which, in many cases, can provide information, advice and even carry out certain procedures. These are the contacts of some of them by Autonomous Community:

All these organisations are external to the Josep Carreras Foundation.

STATE

• AMILO (Asociación Española de Amiloidosis)
• ACLIF (Asociación para la cura del linfoma folicular)
• AEAL (ASOCIACIÓN ESPAÑOLA DE AFECTADOS POR LINFOMA, MIELOMA y LEUCEMIA)
• AECC (ASOCIACIÓN ESPAÑOLA CONTRA EL CÁNCER). Present in the different provinces and in many municipalities. Contact the nearest branch or call 900 100 036 (24h).
• AELCLES (Agrupación Española contra la Leucemia y Enfermedades de la Sangre)
• CEMMP (Comunidad Española de Pacientes de Mieloma Múltiple) 
• JOSEP CARRERAS LEUKAEMIA FOUNDATION
• FUNDACIÓN SANDRA IBARRA
• GEPAC (GRUPO ESPAÑOL DE PACIENTES CON CÁNCER)
• MPN España (Asociación de Afectados Por Neoplasias Mieloproliferativas Crónicas)

ANDALUCÍA

• AECC (ASOCIACIÓN ESPAÑOLA CONTRA EL CÁNCER). Present in the different provinces and in many municipalities. Contact the nearest branch.
• ALUSVI (ASOCIACIÓN LUCHA Y SONRÍE POR LA VIDA). Sevilla
• APOLEU (ASOCIACIÓN DE APOYO A PACIENTES Y FAMILIARES DE LEUCEMIA). Cádiz

ARAGÓN

• AECC (ASOCIACIÓN ESPAÑOLA CONTRA EL CÁNCER). Present in the different provinces and in many municipalities. Contact the nearest branch.
• ASPHER (ASOCIACIÓN DE PACIENTES DE ENFERMEDADES HEMATOLÓGICAS RARAS DE ARAGÓN)
• DONA MÉDULA ARAGÓN

ASTURIAS

• AECC (ASOCIACIÓN ESPAÑOLA CONTRA EL CÁNCER). Present in the different provinces and in many municipalities. Contact the nearest branch.
• ASTHEHA (ASOCIACIÓN DE TRASPLANTADOS HEMATOPOYÉTICOS Y ENFERMOS HEMATOLÓGICOS DE ASTURIAS)

CANTABRIA

• AECC (ASOCIACIÓN ESPAÑOLA CONTRA EL CÁNCER). Present in the different provinces and in many municipalities. Contact the nearest branch.

CASTILLA LA MANCHA

• AECC (ASOCIACIÓN ESPAÑOLA CONTRA EL CÁNCER). Present in the different provinces and in many municipalities. Contact the nearest branch.

CASTILLA LEÓN

• ABACES (ASOCIACIÓN BERCIANA DE AYUDA CONTRA LAS ENFERMEDADES DE LA SANGRE)
• AECC (ASOCIACIÓN ESPAÑOLA CONTRA EL CÁNCER). Present in the different provinces and in many municipalities. Contact the nearest branch.
• ALCLES (ASOCIACIÓN LEONESA CON LAS ENFERMEDADES DE LA SANGRE). León.
• ASCOL (ASOCIACIÓN CONTRA LA LEUCEMIA Y ENFERMEDADES DE LA SANGRE). Salamanca.

CATALUÑA

• ASSOCIACIÓ FÈNIX. Solsona
• FECEC (FEDERACIÓ CATALANA D’ENTITATS CONTRA EL CÁNCER
• FUNDACIÓ KÁLIDA. Barcelona
• FUNDACIÓ ROSES CONTRA EL CÀNCER. Roses
• LLIGA CONTRA EL CÀNCER COMARQUES DE TARRAGONA I TERRES DE L’EBRE. Tarragona
• MielomaCAT 
• ONCOLLIGA BARCELONA. Barcelona
• ONCOLLIGA GIRONA. Girona
• ONCOLLIGA COMARQUES DE LLEIDA. Lleida
• ONCOVALLÈS. Vallès Oriental
• OSONA CONTRA EL CÀNCER. Osona
• SUPORT I COMPANYIA. Barcelona
• VILASSAR DE DALT CONTRA EL CÀNCER. Vilassar de Dalt

VALENCIAN COMMUNITY

• AECC (ASOCIACIÓN ESPAÑOLA CONTRA EL CÁNCER). Present in the different provinces and in many municipalities. Contact the nearest branch.
• ASLEUVAL (ASOCIACIÓN DE PACIENTES DE LEUCEMIA, LINFOMA, MIELOMA Y OTRAS ENFERMEDADES DE LA SANGRE DE VALENCIA)

EXTREMADURA

• AECC (ASOCIACIÓN ESPAÑOLA CONTRA EL CÁNCER). Present in the different provinces and in many municipalities. Contact the nearest branch.
• AFAL (AYUDA A FAMILIAS AFECTADAS DE LEUCEMIAS, LINFOMAS; MIELOMAS Y APLASIAS)
• AOEX (ASOCIACIÓN ONCOLÓGICA EXTREMEÑA)

GALICIA

• AECC (ASOCIACIÓN ESPAÑOLA CONTRA EL CÁNCER). Present in the different provinces and in many municipalities. Contact the nearest branch.
• ASOTRAME (ASOCIACIÓN GALLEGA DE AFECTADOS POR TRASPLANTES MEDULARES)

BALEARIC ISLANDS

• ADAA (ASSOCIACIÓ D’AJUDA A L’ACOMPANYAMENT DEL MALALT DE LES ILLES BALEARS)
• AECC (ASOCIACIÓN ESPAÑOLA CONTRA EL CÁNCER). Present in the different provinces and in many municipalities. Contact the nearest branch.

CANARY ISLANDS

• AECC (ASOCIACIÓN ESPAÑOLA CONTRA EL CÁNCER). Present in the different provinces and in many municipalities. Contact the nearest branch.
• AFOL (ASOCIACIÓN DE FAMILIAS ONCOHEMATOLÓGICAS DE LANZAROTE)
• FUNDACIÓN ALEJANDRO DA SILVA

LA RIOJA

• AECC (ASOCIACIÓN ESPAÑOLA CONTRA EL CÁNCER). Present in the different provinces and in many municipalities. Contact the nearest branch.

MADRID

• AECC (ASOCIACIÓN ESPAÑOLA CONTRA EL CÁNCER). Present in the different provinces and in many municipalities. Contact the nearest branch.
• AEAL (ASOCIACIÓN ESPAÑOLA DE LEUCEMIA Y LINFOMA)
• CRIS CONTRA EL CÁNCER
• FUNDACIÓN LEUCEMIA Y LINFOMA

MURCIA

• AECC (ASOCIACIÓN ESPAÑOLA CONTRA EL CÁNCER). Present in the different provinces and in many municipalities. Contact the nearest branch.

NAVARRA

• AECC (ASOCIACIÓN ESPAÑOLA CONTRA EL CÁNCER). Present in the different provinces and in many municipalities. Contact the nearest branch.

BASQUE COUNTRY

• AECC (ASOCIACIÓN ESPAÑOLA CONTRA EL CÁNCER). Present in the different provinces and in many municipalities. Contact the nearest branch.
• PAUSOZ-PAUSO. Bilbao

AUTONOMOUS CITIES OF CEUTA AND MELILLA

• AECC CEUTA (ASOCIACIÓN ESPAÑOLA CONTRA EL CÁNCER)
• AECC MELILLA (ASOCIACIÓN ESPAÑOLA CONTRA EL CÁNCER)

We also invite you to follow us through our main social media (Facebook, Twitter and Instagram) where we often share testimonies of overcoming this disease.

If you live in Spain, you can also contact us by sending an e-mail to imparables@fcarreras.es so that we can help you get in touch with other people who have overcome this disease.