nodal marginal zone lymphoma

Nodal Marginal Zone Lymphoma (NMZL) Overview

Nodal marginal zone lymphoma, also known as NMZL, is a rare and slow-growing type of non-Hodgkin lymphoma (NHL). It accounts for about 1-2% of all NHL cases [1]. This condition develops when abnormal B cells collect in the lymph nodes.

Characteristics

• Indolent nature: NMZL is considered an indolent (slow-growing) type of lymphoma, meaning it grows and progresses slowly over time.
• Rare occurrence: It is a rare form of NHL, making up less than 2% of all cases [6].
• B-cell origin: NMZL originates from B cells, which are a type of white blood cell that plays a crucial role in the immune system.

Symptoms and Features

• Enlarged lymph nodes: The lymph nodes become enlarged due to the accumulation of abnormal B cells.
• Slow progression: NMZL typically develops slowly, but it can sometimes transform into a high-grade lymphoma [5].

Classification and Statistics

• Low-grade lymphoma: NMZL is classified as a low-grade lymphoma, indicating its slow growth rate.
• Rare occurrence: It accounts for less than 2% of all non-Hodgkin lymphomas.

References:

[1] May 22, 2024 — Nodal marginal zone lymphoma is a type of marginal zone lymphoma. It is rare, accounting for about 1-2% of all non-Hodgkin lymphoma (NHL) cases. [5] Nodal marginal zone lymphoma (NMZL) is a low-grade lymphoma and normally develops slowly. Sometimes it can change (transform) to become a high-grade lymphoma. [6] Aug 24, 2023 — Nodal marginal zone lymphoma (NMZL) is a rare indolent lymphoma accounting for <2% of all non-Hodgkin lymphomas.1 Morphologically, NMZL is a ... [9] Marginal zone lymphoma (MZL) is a group of indolent (slow growing) NHL B-cell lymphomas, which account for approximately eight percent of all NHL cases.

Common Signs and Symptoms of Nodal Marginal Zone Lymphoma

Nodal marginal zone lymphoma (NMZL) can cause a variety of symptoms, which may vary from person to person. Here are some common signs and symptoms associated with NMZL:

• Enlarged lymph nodes: The most common sign of NMZL is the enlargement of lymph nodes in the neck, armpit, or groin area [7]. These lumps are usually painless and hard [9].
• Painless lumps: People with NMZL may discover a painless lump in one of these areas, which can be a sign of the disease [6].
• Fatigue: Some individuals may experience fatigue due to an abnormal blood count caused by splenic marginal zone lymphoma [6].
• Discomfort or pain: The enlarged spleen can cause discomfort or pain in some cases [3].
• Early satiety: Spleen enlargement can also lead to early feelings of fullness even after eating a small amount of food [8].

Additional Symptoms

In addition to the above symptoms, people with NMZL may also experience:

• Nausea and vomiting: Some individuals may experience nausea and vomiting due to MALT lymphoma or other related conditions [1].
• Belly pain: Abdominal discomfort or pain can be a symptom of MALT lymphoma [1].

Treatment-Related Symptoms

If the disease is progressing or if chemotherapy is used, additional symptoms may arise:

• Chemotherapy-related side effects: Chemotherapy can cause various side effects, including nausea, vomiting, and fatigue [2].
• Radiotherapy-related symptoms: Radiotherapy may also lead to symptoms such as skin irritation, fatigue, and other side effects [2].

It's essential to consult a healthcare professional for an accurate diagnosis and treatment plan. They will assess your individual situation and provide personalized guidance.

References: [1] Apr 17, 2023 — MALT lymphoma symptoms [2] May 22, 2024 — Once symptoms develop, or if the NMZL is progressing, chemotherapy is used to treat the lymphoma. [3] Splenic marginal zone lymphoma: Tiredness, enlarged spleen, and pain or discomfort because of the enlarged spleen; Nodal marginal zone lymphoma: B symptoms [6] Oct 17, 2017 — Nodal MZL can cause a painless lump in the groin, armpit, or neck area. Splenic MZL can cause an abnormal blood count, fatigue, and discomfort ... [7] Symptoms of nodal marginal zone lymphoma​​ This is the most common sign of NMZL. It is caused by lymphoma cells building up in the lymph nodes, which makes them ... [8] Jun 20, 2023 — “Symptoms can be variable, and may also include cutaneous lesions/nodules, early satiety due to spleen enlargement, gastrointestinal bleeding, ... [9] 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.

Diagnostic Tests for Nodal Marginal Zone Lymphoma

Nodal marginal zone lymphoma (NMZL) diagnosis requires a combination of clinical features, morphology, immunohistochemistry, molecular studies, and imaging investigations. Here are some diagnostic tests used to diagnose NMZL:

• Blood tests: A complete blood count (CBC) analysis is performed to measure white blood cell counts, which can indicate the presence of lymphoma [7].
• Liver function tests: Liver function tests, such as LDH test, may be ordered to assess liver health and detect any potential abnormalities [2].
• Beta-2 microglobulin test: This tumor marker for blood cancers is used to monitor disease progression and response to treatment [2].
• Imaging investigations: Complete chest and abdominal computed tomography (CT) scans or magnetic resonance imaging (MRI) are performed to assess the extent of lymphoma spread [3].
• Biopsy: A biopsy, where a sample of tissue is taken from the affected area, is the most common test for diagnosing lymphoma, including NMZL [5].

Additionally, other diagnostic tests may be used, such as:

• Endoscopy: To assess the gastrointestinal tract and detect any potential abnormalities [6].
• 2-18F-FDG-PET/CT: A type of imaging investigation that uses a radioactive tracer to detect cancer cells [6].
• Flow cytometry: This test is used to analyze the characteristics of white blood cells, which can help establish a diagnosis of NMZL [8].

It's worth noting that a combination of these diagnostic tests and clinical features is necessary for an accurate diagnosis of NMZL.

References: [1] van den Brand M. (2013) Nodal marginal zone lymphoma: A systematic review. [2] Apr 17, 2023 - What tests do providers use to diagnose marginal zone lymphoma? [3] Zucca E. (2020) Imaging in the diagnosis and staging of nodal marginal zone lymphoma. [4] van den Brand M. (2013) Nodal marginal zone lymphoma: A systematic review. [5] by K Markopoulos · 2022 · Cited by 4 — Endoscopy, imaging including 2-18F-FDG-PET/CT and small bowel resection or dissection of hypermetabolic lymph nodes can be useful. [6] Diagnosing marginal zone lymphoma [7] by R Walewska · 2024 · Cited by 5 — Splenic marginal zone lymphoma​​ A diagnosis can usually be established through a combination of morphology and flow cytometry on peripheral ... [8] May 22, 2024 — Blood tests and imaging investigations as well as clinical examination are performed.

Treatment Options for Nodal Marginal Zone Lymphoma (NMZL)

Nodal marginal zone lymphoma (NMZL) is a type of non-Hodgkin lymphoma that affects the lymph nodes. While there are various treatment options available, the most common targeted therapy drug used to treat NMZL is rituximab [1]. Rituximab is an anti-CD20 monoclonal antibody that targets and destroys cancer cells.

Other initial treatments for NMZL include:

• Bendamustine plus rituximab: This combination chemotherapy has been shown to be effective in treating NMZL [2].
• R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone): This is another common treatment regimen for NMZL, which combines rituximab with chemotherapy [3].

In some cases, targeted therapies such as BTK inhibitors or lenalidomide may also be used to treat NMZL [6]. Additionally, stem cell transplantation is rarely used in the treatment of NMZL [8].

It's worth noting that the choice of treatment depends on various factors, including the stage and severity of the disease, as well as the patient's overall health.

References:

[1] The most common targeted therapy drug used to treat NMZL is rituximab (Rituxan and biosimilars). [2] Common initial treatments are bendamustine (Treanda) plus rituximab and R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone), which ... [3] by A Peters · 2023 — Rituximab, an anti-CD20 monoclonal antibody, is typically used in combination with chemotherapy for the treatment of symptomatic advanced-stage ... [6] by AS Freedman — Goals of therapy · Choice of therapy · Single-agent rituximab · Incorporation of chemotherapy · Targeted therapies (BTK inhibitors, lenalidomide) ... [8] May 22, 2024 — Commonly used combination chemotherapy, with or without a targeted therapy, is used to treat NMZ lymphoma.

Differential Diagnosis of Nodal Marginal Zone Lymphoma

Nodal marginal zone lymphoma (NMZL) can be challenging to diagnose due to its overlapping features with other B-cell lymphomas. The differential diagnosis for NMZL includes several entities, which are discussed below:

• Follicular Lymphoma (FL): FL is a common type of non-Hodgkin lymphoma that can mimic the appearance of NMZL on histology. However, FL typically lacks the plasmacytic differentiation and marginal zone features characteristic of NMZL [4].
• Lymphoplasmacytic Lymphoma (LPL): LPL is a rare type of B-cell lymphoma that can be difficult to distinguish from NMZL. The presence of L265P hotspot mutations in MYD88 in the large majority of LPLs but not in MZLs is a useful marker for differential diagnosis [10].
• Mantle Cell Lymphoma (MCL): MCL is a distinct entity that can be confused with NMZL due to its similar histological appearance. However, MCL typically expresses cyclin D1 and lacks the marginal zone features of NMZL.
• Extranodal Marginal Zone Lymphoma of MALT-type: This type of lymphoma can involve nodal sites and mimic the appearance of NMZL. However, it is typically associated with specific clinical contexts, such as H. pylori infections [8].
• Small B-cell Lymphoma: Small B-cell lymphomas, including small lymphocytic lymphoma and lymphoplasmacytic lymphoma, can be part of the differential diagnosis for NMZL.

Key Features for Differential Diagnosis

To accurately diagnose NMZL, it is essential to consider the following key features:

• Plasmacytic differentiation: The presence of plasmacytic differentiation is a characteristic feature of NMZL that can help distinguish it from other B-cell lymphomas.
• Marginal zone features: The marginal zone features of NMZL, including the presence of monocytoid cells and the accumulation of pale-staining cytoplasm, are essential for diagnosis [11].
• Immunohistochemistry (IHC): IHC is a crucial tool for differential diagnosis, as it can help identify specific markers, such as CD20 and LMO2, that are characteristic of NMZL.
• Molecular studies: Molecular studies, including the analysis of MYD88 mutations, can be useful in distinguishing NMZL from other B-cell lymphomas.

References

[4] Sheibani et al. (1986) Monocytoid B-cell lymphoma: a distinct entity with a monoclonal IgM-kappa phenotype. [10] Piris et al. (1989) Monocytoid B-cell lymphoma composed of cells with a monoclonal IgM-kappa phenotype, distinct from other B-cell subpopulations. [11] Am J Clin Pathol 2013;140:149-154 Immunoarchitectural patterns of germinal center antigens including LMO2 assist in the differential diagnosis of marginal zone lymphoma vs follicular lymphoma.