Primary mediastinal B-cell lymphoma (PMBCL) is a rare type of non-Hodgkin lymphoma that originates in the mediastinum, the area in the chest between the lungs. This type of lymphoma is characterized by the presence of large, abnormal B-cells in the lymphatic tissue of the mediastinum. The disease often presents with symptoms such as chest pain, cough, shortness of breath, and superior vena cava syndrome due to the mass effect of the tumor in the chest.
Why Do People Get Primary Mediastinal B-Cell Lymphoma?
The exact cause of PMBCL is not fully understood. However, it is believed to be related to genetic mutations in B-cells (the cells in the body that produce antibodies to fight infections). These mutations cause the B-cells to grow and divide uncontrollably, leading to the formation of a tumor in the mediastinum. Some studies suggest that certain genetic syndromes, exposure to certain chemicals or radiation, and certain viral infections may increase the risk of developing this type of lymphoma. It's important to note that most people who develop PMBCL have no known risk factors.
Who Gets Primary Mediastinal B-Cell Lymphoma?
PMBCL is a rare subtype of aggressive B cell lymphoma. It constitutes 2%–3% of all cases of non‐Hodgkin lymphoma (NHL). This disease appears most often in children and young adults with B-cell lymphoma, with a slight predominance in females. The cause of PMBCL is unknown. However, people who have DNA repair defects or immune deficiencies, or those who take immunosuppressive medications are at higher risk for developing this type of cancer.
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- How Is Primary Mediastinal B-Cell Lymphoma Staged And Classified?
- What Are Treatments For Primary Mediastinal B-Cell Lymphoma?