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marginal zone lymphoma
ICD-10 Codes
Description
What is Marginal Zone Lymphoma?
Marginal zone lymphoma (MZL) is a type of non-Hodgkin lymphoma that affects the lymphatic system, which includes lymph nodes, organs, and other structures throughout the body. It is characterized by its slow growth and development in certain areas of lymph tissue, known as marginal zones.
Types of Marginal Zone Lymphomas
There are three main types of MZL:
- Extranodal MZL: This type develops outside of the lymph nodes, often in the stomach or small intestine.
- Nodal MZL: This type occurs within the lymph nodes themselves.
- Splenic MZL: This type affects the spleen.
Causes and Risk Factors
The exact cause of MZL is not fully understood, but it is believed to be related to chronic inflammation or random errors in DNA. Certain factors may increase a person's risk of developing MZL, including:
- Age: MZL is more common in older adults.
- Sex: Men are slightly more likely to develop MZL than women.
- Family history: A person with a family history of lymphoma or other cancers may be at higher risk.
Symptoms
The symptoms of MZL can vary depending on the type and location of the disease. Common symptoms include:
- Enlarged lymph nodes
- Fatigue
- Weight loss
- Abdominal pain (if the disease affects the stomach or small intestine)
Treatment Options
Treatment for MZL typically involves a combination of therapies, including:
- Watchful waiting: Monitoring the disease to see if it progresses.
- Chemotherapy: Using medications to kill cancer cells.
- Radiation therapy: Targeting radiation to specific areas of the body affected by the disease.
- Surgery: Removing affected lymph nodes or organs.
Prognosis
The prognosis for MZL varies depending on the type and stage of the disease. In general, MZL is considered an indolent (slow-growing) cancer, which means it may not progress rapidly. However, if left untreated, MZL can eventually lead to more serious complications.
References:
- [1] Marginal zone lymphoma is a rare type of non-Hodgkin lymphoma that develops from chronic inflammation or because of a random error in DNA.
- [3] Marginal zone lymphomas are types of slow-growing (low-grade) non-Hodgkin lymphomas that develop from B cells.
- [9] An indolent (slow-growing) type of B-cell non-Hodgkin lymphoma that begins forming in certain areas (the marginal zones) of lymph tissue.
- [8] MZL are a group of indolent B-cell lymphomas with considerable heterogeneity in terms of clinical presentation, biology, etiology and therapeutic approaches.
Additional Characteristics
- A type of non-Hodgkin lymphoma that affects the lymphatic system.
- Characterized by its slow growth and development in certain areas of lymph tissue, known as marginal zones.
- There are three main types: Extranodal MZL, Nodal MZL, and Splenic MZL.
- Causes include chronic inflammation or random errors in DNA.
- Symptoms include enlarged lymph nodes, fatigue, weight loss, and abdominal pain.
- Treatment options involve watchful waiting, chemotherapy, radiation therapy, and surgery.
- MZL is considered an indolent cancer with varying prognosis depending on the type and stage of the disease.
Signs and Symptoms
Marginal zone lymphoma (MZL) is a type of cancer that affects the immune system, specifically the lymphatic system. The signs and symptoms of MZL can vary depending on the location and extent of the disease.
Common Signs and Symptoms:
- Unexplained weight loss [4]
- Chest or belly pain [4]
- Fever without having an infection [6]
- Night sweats [6]
- Skin rash [4]
- Fatigue [5]
In some cases, MZL can also cause more specific symptoms, such as:
- Swollen lymph nodes in the neck, armpit, or groin [8][9]
- Painless and hard lumps in the neck, armpit, or groin due to swollen lymph nodes [9]
- Anaemia (a shortage of red blood cells), which can make you feel tired or short of breath [7]
- Thrombocytopenia (a low platelet count), which can increase the risk of bleeding [7]
It's essential to note that these symptoms can also be caused by other conditions, and a proper diagnosis can only be made by a healthcare professional through a combination of physical examination, medical history, laboratory tests, and imaging studies.
References:
[4] Oct 17, 2017 — What are the symptoms? · fever without an infection · night sweats · unexplained weight loss · skin rash · chest or abdominal pain · tiredness. [5] Jun 20, 2023 — What are the Symptoms of MZL? · Swollen lymph nodes · Chest pain or problems breathing · Fatigue · Abdominal pain. [6] Oct 17, 2017 — What are the symptoms? · fever without an infection · night sweats · unexplained weight loss · skin rash · chest or abdominal pain · tiredness. [7] Jul 14, 2023 — They'll feel stomach pain or experience vomiting. Or, if it's affecting the skin, the patient will have a rash. [8] Sep 29, 2021 — Painless and hard lumps in the neck, armpit or groin that don't go away are common indicators of NMZL due to the lymph nodes swelling. [9] Aug 10, 2022 — Swollen lymph nodes: This is often the first sign of MZL. The lymph nodes in the neck, armpit, or groin may become enlarged and may or may not be painful.
Additional Symptoms
- Skin rash
- Fever without having an infection
- Swollen lymph nodes in the neck, armpit, or groin
- Painless and hard lumps in the neck, armpit, or groin due to swollen lymph nodes
- Anaemia (a shortage of red blood cells)
- Thrombocytopenia (a low platelet count)
- weight loss
- chest pain
- night sweats
- fatigue
Diagnostic Tests
Diagnostic Tests for Marginal Zone Lymphoma
Marginal zone lymphoma (MZL) diagnosis involves a series of tests and procedures to confirm the presence of this type of non-Hodgkin lymphoma.
- Imaging tests: Complete chest and abdominal computed tomography (CT) scan or magnetic resonance imaging (MRI) are recommended for imaging purposes [1].
- Biopsy: A biopsy of an involved nodal or extranodal site demonstrating a clonal B-cell infiltrate with CD5 and CD10 positivity is crucial for securing the diagnosis [4].
- Blood tests: Blood tests, including complete blood count (CBC) analysis that measures white blood cells, are used to check for viral and bacterial infections [6].
- Bone marrow tests: Bone marrow tests may be performed to assess the extent of disease spread.
- Flow cytometry: Flow cytometry on peripheral blood or bone marrow samples can help identify specific B-cell markers [9].
Additional Diagnostic Tools
- FISH panel: A FISH (Fluorescence In Situ Hybridization) panel is designed to detect common and prognostically-significant abnormalities in MZL [7].
- Imaging services: Imaging services such as MRI scans, CT scans, X-rays, ultrasounds & screening mammograms are available at various medical facilities, including Novant Health Imaging Museum and Carolinas Imaging Services (CIS) [10][11][12].
References
[1] Zucca E. (2020). Marginal zone lymphoma: a review of the literature. Cited by 299.
[4] Cheah CY. (2023). Diagnosis of marginal zone lymphoma. Cited by 11.
[6] Diagnosing marginal zone lymphoma. Blood tests to check for viral and bacterial infections.
[7] Swerdlow SH, et al. (2008). FISH panel for detecting abnormalities in MZL.
[9] Walewska R. (2024). Splenic marginal zone lymphoma diagnosis through morphology and flow cytometry.
Note: The above information is based on the search results provided and may not be an exhaustive list of diagnostic tests for marginal zone lymphoma.
Additional Diagnostic Tests
- Blood tests
- Biopsy
- Imaging tests
- Imaging services
- Bone marrow tests
- Flow cytometry
- FISH panel
Treatment
Treatment Options for Marginal Zone Lymphoma
Marginal zone lymphoma (MZL) is a type of cancer that affects the immune system, and treatment options vary depending on the stage and severity of the disease.
- First-line treatment: Single-agent rituximab has been found to be effective as first-line treatment for MZL [2]. Rituximab is a monoclonal antibody that targets CD20 proteins on B cells.
- Second-line treatment: For patients who have failed antibiotic therapy or have contraindications, rituximab is recommended as a second-line treatment option [3].
- Other treatment options: Treatment options for MZL also include splenectomy (removal of the spleen), chemoimmunotherapy (combination of chemotherapy and immunotherapy), and single-agent rituximab [4]. However, none of these treatments have been shown to have a clear survival benefit over others.
- Targeted therapy: In recent years, novel agents such as lenalidomide, Bruton's tyrosine kinase (BTK) inhibitors, and phosphoinositide 3-kinase (PI3K) inhibitors have been introduced for the treatment of MZL [7].
- Biologics: Patients with splenic marginal zone lymphoma often receive biologic therapy, such as rituximab, in combination with chemotherapy [8].
Treatment for Advanced Stage Nodal MZL
For patients with advanced stage nodal MZL, the most likely treatment is antibody therapy (such as rituximab) combined with chemotherapy, also known as chemoimmunotherapy [9]. This approach has shown promise in improving outcomes for these patients.
It's essential to note that treatment decisions should be made on an individual basis and under the guidance of a healthcare professional. The specific treatment plan will depend on various factors, including the stage and severity of the disease, overall health, and personal preferences.
Recommended Medications
- Rituximab
- Phosphoinositide 3-kinase (PI3K) inhibitors
- Chemoimmunotherapy
- Splenectomy
- Antibody therapy
- tyrosine kinase inhibitor
- lenalidomide
💊 Drug information is sourced from ChEBI (Chemical Entities of Biological Interest) database. Always consult with a healthcare professional before starting any medication. Click on any medication name for detailed information.
Differential Diagnosis
Differential Diagnosis of Marginal Zone Lymphoma
Marginal zone lymphoma (MZL) is a type of non-Hodgkin lymphoma that can be challenging to diagnose due to its overlapping features with other conditions. The differential diagnosis for MZL includes:
- Cutaneous pseudolymphoma: A benign condition characterized by
Additional Differential Diagnoses
Additional Information
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