Understanding Extranodal Marginal Zone Lymphoma

Introduction:

About five percent of adult lymphomas are extranodal marginal zone lymphoma. They occur at extranodal sites, followed by inflammation. They can be cured by local excision.

What Is Extranodal Marginal Zone Lymphoma?

EMZL is a cancer of the lymphatic system or lymph nodes. They are the first line immune system of the body. Marginal cell lymphomas slowly progress to cancer. It is a type of non-Hodgkins B-cell type lymphoma. Depending on the spread of infection, it is further classified into three sub-types:

i.) Extranodal Marginal Zone Lymphoma (EMZL): If the spread of hemostasis occurs in the mucosal region, it is called mucosa-associated lymphoid tissue (MALT). It produces inflammatory conditions like Sjogren’s syndrome and Helicobacter pylori gastritis in the stomach. These are the most common type of lymphoma. EMZL are extranodal lymphomas mainly affecting lymph nodes. MALT occurs in organs other than lymph nodes like skin, stomach, thyroid, lungs, bowel, and areas around the eyes. They usually respond well to the treatment.

ii.) Splenic Marginal Zone Lymphoma (SMZL): These are rarest form of lymphoma. They occur in blood, bone marrow, and spleen. However, the treatment and prognosis are questionable due to its late symptom arousal.

iii.) Nodal Marginal Zone Lymphoma (NMZL): These are slow growing and the rarest forms of lymphomas occurring in the human body.

What Causes Extranodal Marginal Zone Lymphoma?

Mutations in genes cause these infections. They can be inherited through parents and develop as lymphoma affecting children. Gene mutations are caused by locally induced stress and autoimmune disorders. No specific gene has been linked to mutations of extranodal marginal zone lymphoma. Other factors involved are:

• Chronic Hepatitis C Infection: Prolonged or recurrent infection of hepatitis C creates stress in the lining of the spleen mucosa. This makes splenic marginal or nodal marginal zone lymphomas.

• Autoimmune Disorders: Autoimmune conditions like lupus and rheumatoid arthritis occur outside the stomach. This also affects the mucosal linings, and hence MALT conditions arise.

• Bacterial Infections: Increased bacterial proliferation, such as Helicobacter pylori, causes gastritis and MALT lymphomas of the stomach. Other bacterias like Campylobacter jejuni and Chlamydophila psittaci are also bacteria seen in the stomach, which also gives rise to lymphomas of the stomach.

What Are the Symptoms Associated With Extranodal Marginal Zone Lymphomas?

Most of the conditions show late symptoms or no symptoms at all. Then depending on the spread and location of lymphoma, symptoms start showing. Such as:

• Weight loss.

• Fever.

• Night sweats.

• Indigestion.

• The fullness of the stomach.

• Pain in the left abdominal region.

• Nausea and vomiting.

• Tiredness.

• Enlarged spleen.

• Enlarged lymph nodes.

• Skin rash.

What Are the Risk Factors Associated With Extranodal Marginal Zone Lymphoma?

Marginal zone lymphomas can cause conditions such as non-Hodgkins lymphoma. These are the rarest forms of blood cancer. Other risk factors include:

• Senile patients or old patients.

• Most commonly seen in males than females.

• Weakened immune system.

• Infections are caused by the Epstein-Barr virus or HTLV-1 virus.

How to Test for Marginal Zone Lymphomas?

Based on symptoms, the tests can be performed. Consulting with a physician, performing local examinations like palpitating the enlarged lymph nodes, and checking for swelling or enlarged spleen manifestations are of utmost importance. If suspected, the doctor will conduct the following tests to confirm the diagnosis.

• Complete Blood Count: Elevated levels of neutrophils, basophils, megakaryocytes, and eosinophils can indicate lymphoma.

• Microbiology Study: To check for the presence of bacterial infections like Campylobacter jejuni, Chlamydophila psittaci, and Helicobacter pylori.

• Blood Tests: Check for hepatitis B and C virus and human immunodeficiency (HIV) virus.

• Bone Marrow Aspiration Biopsy: Samples are collected from bone marrow tissue to study under the microscope and check for lymphoma.

• Computed Tomography: To check for the extent of the spread of lymphoma and metastasis.

• Fluorescence in-Situ Hybridisation (FISH): This chromosomal detection test helps in diagnosing the translocation of chromosomes, such as MALT1 and BCL10 genes. This leads to the activation of the NF-kB signaling pathway.

What Are the Treatments Available to Cure Extranodal Marginal Zone Lymphomas?

Various treatment modalities are present to treat lymphoma. Treatment is determined depending on the symptoms occurring and the severity of the condition. Below are some of the treatments available:

• Chemotherapy: This is treated by killing cancer-causing cells using chemicals. This is done by giving anti-chemotherapeutic drugs like Abraxane, Cytosar U, and Nilotinib drugs.

• Radiation Therapy: High radiation doses are given to kill cancer-causing cells. This is given in dosages and a stipulated number of doses.

• Oncogene Targeted Therapy: Targeted therapies like Rituximab are given specifically to cancer sites.

• Antibiotic Therapy: This is used in patients suffering from MALT lymphomas of the stomach. Drugs such as Flagyl, Tetracycline, and Amoxicillin are given.

• Antiviral Therapy: It is done in patients with hepatitis B and C infections. Drugs for treating hepatitis C like Epcluza, Ribavirin, and combination drugs like Ledipasvir-Sofosbuvir are given.

• Surgical Procedures: Removal of spleen or splenectomy and removal of lymph nodes or lymphadenectomy by laparoscopic procedures are done. One or more lymph nodes are removed based on the need.

• Palliative Care: This is done during late diagnosis when the spread of the tumor has extended beyond care. Patients experience intense pain, so palliative care is given to eliminate the pain.

• Stem Cell Transplant: The stem cells from a donor are collected and preserved under freezing conditions. This is later injected into the patient at intervals of time.

What Is the Differential Diagnosis for Marginal Zone Lymphomas?

Other conditions mimicking marginal zone lymphomas are:

• Marginal zone hyperplasia.

• Diffuse large B-cell lymphomas (DLBCL).

• Small cell lymphomas.

Conclusion:

Early detection of marginal zone lymphoma gives way to earlier treatment and recovery. However, the prognosis gives a three-year survival rate. Unfortunately, some risk factors, like a weakened immune system, old age, and other comorbid conditions, make recovery difficult.