Definition. Marginal zone lymphomas are indolent lymphomas that arise from memory B cells in the marginal zone of lymphoid tissue. They include splenic marginal zone lymphoma, nodal marginal zone lymphoma, and extranodal marginal zone lymphoma .
Most patients with nodal marginal zone lymphoma present with advanced stage disease and are not likely to achieve cure, even with aggressive chemotherapy regimens.
Subsequently, question is, what causes marginal zone lymphoma? The exact cause of nodal and splenic MZL is unknown. In the case of MALT, inflammation due to an infection may be responsible. The disease can develop if you've been infected with H.
Marginal Zone Lymphoma. 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. The average age at diagnosis is 60 years, and it is slightly more common in women than in men.
The marginal zone is the region at the interface between the non-lymphoid red pulp and the lymphoid white-pulp of the spleen.
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Diffuse large B cell lymphoma (DLBCL) is the most common aggressive subtype. It affects about 30 percent of people with NHL in the United States.
What is the prognosis like? Hodgkin lymphoma is considered one of the most treatable cancers, with more than 90 percent of patients surviving more than five years. Most patients with Hodgkin lymphoma live long and healthy lives following successful treatment.
More than 90 out of 100 people (more than 90%) survive for 5 years or more after diagnosis. Between 75 and 90 out of 100 people (between 75 and 90%) will survive for 5 years or more after they're diagnosed. Even if Hodgkin lymphoma comes back, it can often be treated successfully again.
The marginal zone is at the edge of the area of lymphoid tissue and is where B cells are normally found (lymphoid tissue is part of the immune system, for example the lymph nodes or spleen). Other types of marginal zone lymphomas are MALT lymphoma (extranodal marginal zone lymphoma) and splenic marginal zone lymphoma.
The second most common subtype of NHL, follicular lymphoma (FL), grows slowly, with lymph nodes doubling in size approximately every six to 12 months, and patients often get diagnosed a year or later after they first noted an enlarged lymph node.
The prognosis for survivors with any subtype of indolent B-cell NHL improved slightly, up to 80–90%, with each additional year of survival after diagnosis. For patients with DLBCL CRS improved markedly during the first year after diagnosis (from 48% at diagnosis to 71%) and thereafter gradually to 95% after 16 years.
Patients with SMZL often have an indolent course with median life expectancy greater than 10 years. However, about a quarter of patients have a more aggressive course, with disease progression and death within the first few years after diagnosis (Arcaini et al, 2006).
Once their lymphoma is in remission, some people are offered maintenance antibody treatments, eg rituximab or a newer antibody like obinutuzumab. You have these treatments once every 2–3 months for up to 2 years to keep your lymphoma in remission.
Lymphoma most often spreads to the liver, bone marrow, or lungs. Stage III-IV lymphomas are common, still very treatable, and often curable, depending on the NHL subtype. Stage III and stage IV are now considered a single category because they have the same treatment and prognosis.
Stages III, IV, and most stage II bulky lymphomas If the lymphoma shrinks, a total of 6 cycles of chemo plus rituximab is usually given. Other options for initial treatment include rituximab alone or chemo alone (either one or several drugs).
For many people with non-Hodgkin lymphoma, treatment can destroy the lymphoma. For some people, the lymphoma may never go away completely. These people may get regular treatments with chemo, radiation, or other therapies to help keep the lymphoma in check for as long as possible and to help relieve symptoms.
Angioimmunoblastic T-Cell Lymphoma (AITL) is a rare, aggressive type accounting for about seven percent of all patients with T-cell lymphomas in the United States.
Treatment for most patients is chemotherapy (usually 2 to 4 cycles), followed by radiation to the initial site of the disease (ISRT or involved site radiation therapy). Another option is chemotherapy alone (usually for 4 or 6 cycles) in selected patients.
Gray zone lymphoma is a rare type of lymphoma, cancer of a part of the immune system called the lymph system. It is called "gray zone" lymphoma because it has features intermediate between classical Hodgkin lymphoma and diffuse large B-cell lymphoma (DLBCL), but cannot be assigned specifically to either type.
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