What is extranodal marginal zone lymphoma?

Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) is a mature B-cell neoplasm that occurs in a variety of extranodal tissues, including the gastrointestinal tract, lung, oropharynx, salivary gland, ocular adnexa, and skin, often arising in the setting of chronic inflammation or …

How is lymphoma marginal zone diagnosed?

This usually involves having a CT scan. You might also have a PET scan, although this is less common for marginal zone lymphomas than other types of lymphoma. You might have a sample of your bone marrow cells collected (a bone marrow biopsy) to check if you have lymphoma cells in your bone marrow.

Is marginal zone lymphoma High grade?

CONCLUSIONS. Marginal zone lymphomas are typically indolent small B-cell lymphomas, although a subset undergoes high-grade transformation, as defined by the presence of sheets of large atypical cells. Rarely, transformation to other aggressive B-cell lymphomas, such as classic Hodgkin lymphoma, can be seen.

How is ocular MALT lymphoma treated?

Radiation therapy as the initial treatment has been reported to be very effective in MALT lymphoma of the orbit [11-28]. Radiotherapy with a dose range of 25 to 35 Gy seems to be a standard approach because it provides local control and cure for localized orbital lymphoma [11,13,28].

How long can you live with marginal zone lymphoma?

Extranodal marginal zone B-cell lymphomas have a slightly better outcome than the other types. Almost 90 out of 100 people with this type of marginal zone lymphoma (90%) survive for 5 years or more after they are diagnosed.

How long can you live with splenic marginal zone lymphoma?

The median overall survival in most series is about ten years and 70% of the patients can remain treatment free for five years. 17, 36, 53 There is no survival benefit for the association of chemotherapy with splenectomy,17 although some studies report increases in overall response rates.

Does marginal zone lymphoma spread?

Less commonly, it can develop in the small bowel, salivary gland, thyroid gland, tear glands or lungs. These lymphomas tend not to spread beyond the place where they started. This type of marginal zone lymphoma starts within the lymph nodes. It is sometimes called monocytoid B cell lymphoma.

How common is ocular lymphoma?

Intraocular lymphoma is rare, with fewer than 200 cases being reported. This type of lymphoma is estimated to represent 1% of NHLs, 1% of intracranial tumors, and less than 1% of intraocular tumors.

Is eye lymphoma treatable?

Treatment. Ocular lymphomas don’t improve on their own. Because of the nonspecific nature of primary intraocular lymphoma presentation, the condition is a diagnostic challenge. The prognosis for this condition remains poor with a five-year mortality of less than 25%.

Is marginal zone lymphoma curable?

Most patients with nodal marginal zone lymphoma present with advanced stage disease and are not likely to achieve cure, even with aggressive chemotherapy regimens. Moreover, there is little evidence that choice of initial therapy has any impact on survival.

What is stage IV marginal zone lymphoma?

The Ann Arbor staging system is commonly used for Hodgkin’s lymphoma patients. It has also been used for the treatment of NHL, including MZL. The Ann Arbor staging system Stage IV indicates diffuse or disseminated involvement of one or more extra-lymphatic organs, including any involvement of the liver or bone marrow.

What does extranodal mean in lymphoma?

The term extranodal disease refers to lymphomatous infiltration of anatomic sites other than the lymph nodes. Almost any organ can be affected by lymphoma, with the most common extranodal sites of involvement being the stomach, spleen, Waldeyer ring, central nervous system, lung, bone, and skin.

What does lymphoma, B-cell, Marginal zone stand for?

Marginal zone B-cell lymphomas, also known as marginal zone lymphomas (MZLs), are a heterogeneous group of lymphomas that derive from the malignant transformation of marginal zone B-cells. Marginal zone B cells are innate lymphoid cells that normally function by rapidly mounting IgM antibody immune responses to antigens such as those presented by infectious agents and damaged tissues. [2]

What is a marginal zone?

[edit on Wikidata] The marginal zone is the region at the interface between the non-lymphoid red pulp and the lymphoid white-pulp of the spleen. (Some sources consider it to be the part of red pulp which borders on the white pulp, while other sources consider it to be neither red pulp nor white pulp.) A marginal zone also exists in lymph nodes.

What is nodal marginal zone B-cell lymphoma?

Nodal marginal zone B-cell lymphoma is a type of non-Hodgkin lymphoma (NHL). It has also been called monocytoid B cell lymphoma or MZL. Lymphomas are cancers of lymph cells, a type of blood cell. There are two main categories- Hodgkin lymphoma and non-Hodgkin lymphoma (NHL).