What Is Extranodal Marginal Zone B-cell Lymphoma (Malt Lymphoma)?
Extranodal Marginal Zone B-cell Lymphoma is a type of non-Hodgkin lymphoma, which grows slowly. Lymphoma is a type of blood cancer that develops when lymphocytes (white blood cells) excessively multiply and grow out of control. Lymphocytes make up the immune system. In your lymphatic system, they travel across your body assisting you combat infections.
There are two types of lymphocytes, B lymphocytes (B-cells) and T lymphocytes (T –cells). Based on the cell type, Lymphomas can be classified as Hodgkin lymphoma and non-Hodgkin lymphoma. MALT (mucosa-associated lymphoid tissue) lymphoma is a slowly growing non-Hodgkin lymphoma that arises from the B cells. It belongs to a type of marginal zone lymphoma: a lymphoma that forms at the edge of lymphoid tissues (lymphocyte/WBC collections) in a specific area called the marginal zone.
Extranodal marginal zone lymphomas (EMZLs) are marginal zone lymphomas (cancer of white blood cells) that grow in tissues outside of the lymph nodes. These lymphomas occur outside the lymph nodes, for example in the gastrointestinal tract, thyroid, skin, leptomeninges (protective membranes outside the brain), and spinal cord. It occurs more often in the stomach but it may develop in other parts of the body.
Disease Epidemiology
Among the Extranodal margin zone B cell lymphomas, the stomach is the most prevalent (common) extranodal site with the Incidence rate (frequency of disease occurrence) of 3.8, accompanied by eye/adnexa 1.4, lungs, skin, and salivary glands 0.9–1.
Disease Etiology
A recurrent pathogen infection with a genetic predisposition towards lymphoma is the main cause. Following infectious agents and autoimmune disorders with genetic mutation play a role in the development of extranodal B cell lymphoma in different extranodal tissues. These are;
- Repeated infection with Helicobacter pylori (H. Pylori) with a genetic mutation may cause the EMZL of stomach named as Primary Gastric EMZL.
- Sjogren syndrome with some genetic mutations (alterations/changes) is responsible for 4.3% cases of salivary and lacrimal gland lymphomas named as primary salivary gland EMZL (extranodal marginal zone lymphoma.
- Hashimoto Thyroiditis (an autoimmune disease of the thyroid) with some underlying genetic mutations is responsible for 0.5% cases of thyroid lymphomas named as primary thyroid EMZL.
- Similarly, recurrent (repeated) infection with chlamydia psittaci, Borellia burgdorferi, campylobacter jejuni, achromobacter with underlying genetic mutations are responsible for eye, skin, small intestines and lungs EMZL ( MALT Lymphoma) respectively.
Sign and Symptoms
Signs and symptoms vary according to the affected tissue or organ. As MALT lymphoma occurs outside the lymph nodes, it normally does not cause swollen lymph nodes. People with MALT lymphoma experience “B symptoms” very rarely (a combination of unexplained weight loss, night sweats, and fever).
MALT Lymphoma usually categorized into gastric MALT lymphoma (lymphoma which is originated from extranodal marginal zone lymphoid tissues of stomach mucosa) and non-gastric MALT lymphoma on the basis involvement of organs or tissues.
Symptoms of gastric MALT Lymphoma:
Gastric MALT lymphoma occasionally shows symptoms and mostly found on examination when the patient presents with any other complaint. Some vague symptoms are;
- Abdominal pain ( Pain in abdomen/belly)
- Indigestion (pain or discomfort in the upper part of the abdomen or burning behind the chest usually referred to as heart burning).
- Nausea ( a feeling of sickness with an inclination to vomit)
- Vomiting
Symptoms of non- gastric MALT Lymphoma
- MALT lymphoma of the small intestine (bowel) can show signs and symptoms of diarrhea (loose stool), pain in the abdomen (belly), bloating, weight loss, and lethargy.
- MALT lymphoma of eye adnexa (eyelid and lacrimal/tear gland) can produce redness of the eye, a lump or mass at the corner of the eyelid, double vision and ptosis (eyelid drooping).
- MALT lymphoma of the lung is also silent most of the time but rarely can present with the symptoms of chest pain, cough, hemoptysis (coughing up blood), and shortness of breath (difficulty in taking a breath).
- MALT Lymphoma of the salivary gland will show the sign of localized gland swelling (a mass in front of the ear or at jaw).
- MALT lymphoma of the thyroid gland will show signs and symptoms of a lump or a mass in front of neck/throat, difficulty in swallowing, hoarse voice, hypothyroidism (decreased secretion of thyroid hormones which contribute in body metabolism) and sometimes difficulty in breathing.
- MALT lymphoma of the skin may present with skin lesions most typically plaques (a raised patch of skin), papules (raised discolored area of skin with distinct margins) or nodules (hard mass).
Diagnosis and Treatment
Common diagnostic tests and procedures for diagnosing the MALT lymphoma are;
- Imaging techniques like USG, CT-scan, PET-scan, MRI, and X-ray.
- Bone marrow and affected tissue biopsy.
- Immunohistochemistry
- Endoscopy in a case where MALT lymphoma is of gastrointestinal in origin.
- Bronchoscopy where MALT lymphoma is originated from lung tissues.
- A urea breath test or stool antigen test for H.Pylori.
Treatment options include surgical removal of the affected tissue, chemotherapy, immunotherapy, targeted therapy, and radiotherapy. Treatment recommendations vary according to the type, staging of the lymphoma, and general health of the patient.
What support can we give for Extranodal Marginal Zone B-Cell Lymphoma?
Extranodal Marginal Zone B-Cell Lymphoma is a rare cancer, meaning it is not as well known as other forms of cancer. Without a Ribbon is an Australian organisation that provides support for individuals who suffer from rare cancers. We provide a designated platform for Warriors to obtain information specific to their Rare Cancer. We also provide annual opportunities for our Warriors to meet and learn from each other. If you suffer from rare cancer such as Extranodal Marginal Zone B-Cell Lymphoma, we can help and support you through your journey thanks to the generous donations we receive. Click the link below to sign up and become a Warrior today!
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