Mucosa-Associated Lymphoid Tissue Lymphomas (MALTomas) - Symptoms, Diagnosis, and Treatment

What Is Mucosa-Associated Lymphoid Tissue Lymphomas (Maltomas)?

Mucosa-associated lymphoid tissue lymphoma is a subtype of extranodal marginal zone lymphoma, which is the type of non-Hodgkin’s lymphoma. It can be observed in various parts of the body involving gastric mucosa, salivary glands, ocular surface, lungs, thyroid gland, breast, and other internal organs. Marginal zone lymphoma is defined as the lymphomas developed in the edge of the marginal zones of the lymph nodes; there are three different types of marginal zone lymphoma involving mucosa-associated lymphoid tissue lymphoma, nodal marginal zone lymphoma, and splenic marginal zone lymphoma. The term MALT is referred to as pseudo lymphoma. These are slower-growing lymphomas than the other types of non-Hodgkin’s lymphoma.

What Is the Etiology of Mucosa-Associated Lymphoid Tissue Lymphomas (Maltomas)?

Mucosa-associated lymphoid tissue lymphoma is the most common type of marginal zone lymphoma. It originates from the lymphoid tissue influenced by chronic inflammation in the extranodal sites of the body.

• Helicobacter pylori is the most common bacteria causing mucosa-associated lymphoid tissue lymphoma in the stomach.

• Borrelia burgdorferi causes the disorder involving the skin.

• Different bacterias for various sites play as causative agents for mucosa-associated lymphoid tissue lymphoma like Chlamydophila psittaci in the ocular adnexa, Campylobacter jejuni in the small intestine, and Achrompbacter xylosoxidans in the lungs.

• Hepatitis C also leads to mucosa-associated lymphoid tissue lymphoma.

• Some auto immunological disorders and chronic infections like Sjogren syndrome increase the risk of the development of mucosa-associated lymphoid tissue lymphoma.

What Is the Epidemiology of Mucosa-Associated Lymphoid Tissue Lymphomas (Maltomas)?

Mucosa-associated lymphoid tissue lymphomas (MALTomas) are generally associated with non-Hodgkin’s lymphoma. Around 15 - 20% of non-Hodgkin’s lymphoma patients tend to develop mucosa-associated lymphoid tissue lymphomas in males aged above 40 - 50 years.

In males, gastrointestinal involvement is seen whereas, in females, thyroid and salivary glands are more involved.

What Are the Clinical Manifestations of Mucosa-Associated Lymphoid Tissue Lymphomas (Maltomas)?

Mucosa-associated lymphoid tissue lymphoma is a subtype of non-Hodgkin’s lymphoma.

Following are the clinical features of mucosa-associated lymphoid tissue lymphomas -

• High-grade fever.

• Weight-loss

• Fatigue.

• Headache.

• Night sweats.

• Lymphadenopathy (swollen lymph nodes).

• Organ-associated symptoms include chest pain, shortness of breath, abdominal discomfort, petechiae, and eye problems.

What Is the Pathophysiology of Mucosa-Associated Lymphoid Tissue Lymphomas (Maltomas)?

Mucosa-associated lymphoid tissue lymphoma is an indolent type of lymphoma involving B-cell lymphocytes of the marginal zone. The lymph node is considered a secondary lymphoid organ, and the B-cells develop into mature B-cells in the cortex or the external part of the lymph nodes. This releases antibodies into the plasma; other than antigenic stimulation, oncological events and lymphoproliferative changes are also observed, including chromosomal activities. These changes can happen because of some genetic mutation or external factors.

The histopathology of mucosa-associated lymphoid tissue lymphomas has normal morphology with malignant cells present in the outer part of the marginal zone with pale cytoplasm and moderately spread chromatin.

What Are the Investigations for the Diagnosis of Mucosa-Associated Lymphoid Tissue Lymphomas (Maltomas)?

The Mucosa-associated lymphoid tissue lymphoma can be diagnosed by performing the following investigation -

• Recording patient’s history.

• Physical Examination such as inspection, palpation, auscultation, and measuring the vital is very important for the diagnosis of the disorder.

• Genetic analysis is done by sample collection for biopsy and fine needle aspiration.

• Complete blood count - It is a blood test performed to determine the count of blood cells.

• Lactate dehydrogenase test - Lactate dehydrogenase is a type of cell-forming enzyme; the enzyme level increases because of some underlying diseases.

• Beta 2-microglobulin Test - Beta 2 microglobulin is a type of protein present on the walls of the cells, tissues, and organs; the increased amount of protein in blood or urine indicates certain disorders.

• Endoscopy - It is a minor invasive procedure that includes a pipe with a camera that is inserted into the gastrointestinal tract through the mouth; while the patient is under sedatives, the camera shows the condition of the mucosal lining of the tract and helps in making the diagnosis.

• Bronchoscopy - It is a diagnostic procedure involving the pipe with the camera on the tip, and same as endoscopy, it is also inserted into the lungs through the mouth.

• Bone marrow biopsy and aspiration are also advised.

• Computed tomography scan (CT) and positron emission tomography (PET-scan) - Are imaging techniques to diagnose and determine the spread of the disease.

• Other blood tests involving hepatitis C and B tests, antigen tests, liver function tests, and renal function tests are also advised.

What Are the Treatment Modalities for Mucosa-Associated Lymphoid Tissue Lymphomas (Maltomas)?

The mucosa-associated lymphoid tissue lymphoma is a curable disorder, and treatment planning is done depending upon its causative agents.

• Antibiotic therapy - Antibiotic drugs treat mucosa-associated lymphoid tissue lymphoma caused by bacterial infections, and different classes of antibiotic drugs are administered for different bacterial causes.

• Chemotherapy - Intravenous administration of drugs to fight against cancer cells.

• Radiation therapy - Using high-energy X-rays is used for the treatment of malignancy.

• Systemic therapy involves the treatment of underlying diseases.

• Chemo-immunotherapy - Administration of chemotherapy with monoclonal antibodies like Rituximab is indicated.

• Surgical procedure involving removal of the affected part.

What Is the Differential Diagnosis of Mucosa-Associated Lymphoid Tissue Lymphomas (Maltomas)?

The mucosa-associated lymphoid tissue lymphoma is the subtype of non-Hodgkin’s lymphoma; therefore, it can be mistaken for some disorders.

• Reactive lesions - Tumor-like lesions caused by chronic inflammation.

• Nodal or splenic zone marginal lymphoma - These are other types of marginal zone lymphoma.

• Diffuse large B-cell lymphoma.

• Mantle cell lymphoma.

• Follicular lymphoma.

What Is the Prognosis of Mucosa-Associated Lymphoid Tissue Lymphomas (Maltomas)?

The prognosis of mucosa-associated lymphoid tissue lymphoma is good according to the affected patient’s age or by early detection of the disorder and different risks; groups are divided into low, moderate, and high-risk groups.

What Are the Complications in Mucosa-Associated Lymphoid Tissue Lymphoma (Maltomas)?

Mucosa-associated lymphoid tissue lymphoma has related complications involving systemic complications.

• Gastric bleeding.

• Anemia.

• Intestinal or gastric perforation.

• Obstruction.

• Secondary cancer.

• Decreased blood count.

Conclusion

An individual with mucosa-associated lymphoid tissue lymphoma should consult a blood specialist called – a hematologist for diagnosis, treatment planning, and follow-up: lifestyle moderation, healthy eating, and regular exercise. Managing mucosa-associated lymphoid tissue lymphoma is complex; it requires an experienced healthcare professional, including oncologists, pathologists, gastroenterologists, radiologists, and other healthcare workers.