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MALT type extranodal B-cell lymphoma

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Information reviewed by Dr. Rocío Parody Porras, Doctor specialising in Haematology. Member of the Management of REDMO (Bone Marrow Donor Registry) and of the Foundation’s medical team. Barcelona Medical Association (Co. 35205)

MALT-type extranodal non-Hodgkin’s lymphomas are a rare form of indolent (slowly progressive) B-type lymphomas, accounting for about 7-10% of B-type non-Hodgkin’s lymphomas and up to 50% of lymphomas primarily affecting the stomach. ‘Extranodal’ refers to the primary site of presentation of the lymphoma being outside the lymph nodes. MALT is a mucosa-associated lymphoid tissue and is localised in specific areas: mucosa of the eyes, intestine, etc. For example, in Peyer’s patches, regions in the form of clusters of lymphoid tissue that line the inside walls of the small intestine. These are areas of the immune system where there are large numbers of immune cells, especially lymphocytes and dendritic cells. The term “MALT” is an acronym for “mucosa-associated lymphoid tissue”. Most cases occur in adults with an average age of 60 years, with a slight female predominance.

Regarding the causes of MALT lymphoma, its development is usually preceded by a chronic inflammatory process which results in the recruitment of lymphocytes to the site of inflammation forming lymphoid tissue similar to Peyer’s patches. Infection by a bacterium that infects the human gastric epithelium, usually Helicobacter pylori (in 90% of cases), causes MALT tissue to appear in the form of follicular gastritis and, from this, molecular changes may occur that could lead to MALT lymphoma.

Other forms of MALT lymphomas have been linked to other infections such as:

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

Other autoimmune conditions such as Sjögren’s syndrome or Hashimoto’s thyroiditis also present a higher risk of developing MALT lymphomas than in people free of autoimmune pathologies.

Regarding the particular clinical features of MALT lymphoma, patients usually visit a doctor with gastric symptoms: heartburn, haemorrhage, epigastric or abdominal pain. This type of lymphoma usually remains localised, but may eventually spread to other areas such as the lungs, intestines or bone marrow. In 10% of patients with MALT lymphoma, the disease develops into an aggressive lymphoma with a poor prognosis.

As for diagnosis, in addition to the tests common to other non-Hodgkin’s lymphomas (NHL), gastric MALT lymphomas should also be diagnosed by fibrogastroscopy with multiple biopsies from the different gastric regions. It is also important to perform an endoscopic ultrasound study to assess the depth of infiltration by the lymphoma; as this has been directly related to the response to treatment. The search for Helicobacter pylori is mandatory in gastric MALT lymphoma.

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

t(11;18), t(14;18), t(1;14) and t(3;14).

In cases of localised gastric MALT lymphoma, eradication of the bacterium Helicobacter pylori (present in the vast majority of cases) by means of antibiotics leads to disappearance of the lymphoma at microscopic level in most cases, although responses may be slow. Most gastric MALT lymphomas are in the localised stage at the time. The commonly accepted initial therapeutic strategy is antibiotic treatment combined with proton pump inhibitors. The most commonly used treatment regimen is clarithromycin, amoxicillin and omeprazole. There are other rescue regimens for cases in which the germ is resistant to first-line treatment.

If the disease does not resolve or is not limited to the stomach, immunochemotherapy will be necessary, usually based on agents such as chlorambucil, fludarabine, bendamustine or CHOP-type combinations, associated with Rituximab (a monoclonal antibody against the CD20 receptor specifically presented by neoplastic lymphoid cells). Once achieved, remission is stable in 90% of cases. Radiotherapy has also been shown to be effective in this type of lymphoma and can be applied locally to the area affected by the lymphoma.

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%.

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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


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


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

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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.

* In accordance with Law 34/2002 on Information Society Services and Electronic Commerce (LSSICE), the Josep Carreras Leukemia Foundation informs that all medical information available on www.fcarreras.org has been reviewed and accredited by Dr. Enric Carreras Pons, Member No. 9438, Barcelona, ​​Doctor in Medicine and Surgery, Specialist in Internal Medicine, Specialist in Hematology and Hemotherapy and Senior Consultant of the Foundation; and by Dr. Rocío Parody Porras, Member No. 35205, Barcelona, ​​Doctor in Medicine and Surgery, Specialist in Hematology and Hemotherapy and attached to the Medical Directorate of the Registry of Bone Marrow Donors (REDMO) of the Foundation).

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