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Splenic marginal zone lymphoma (SMZL) is a type of marginal zone lymphoma, a cancer made up of B-cells that replace the normal architecture of the white pulp of the spleen. The neoplastic cells are both small lymphocytes and larger, transformed lymphoblasts , and they invade the mantle zone of splenic follicles and erode the marginal zone ...
Splenic marginal zone lymphoma (SMZL) is a low grade lymphoma in which malignant B-cells accumulate in the spleen, bone marrow, and, less commonly, the circulation. While generally an indolent disease, about 5-10% of cases transform into a far more aggressive malignancy, diffuse large B-cell lymphoma. [64]
Similar to B1 B cells, MZ B cells can be rapidly recruited into the early adaptive immune responses in a T cell-independent manner. [9] The MZ B cells are especially well-positioned as the first line of defense against systemic blood-borne antigens that enter the circulation and become trapped in the spleen. [10]
Micrograph showing Hodgkin's lymphoma, a type of B cell lymphoma that is usually considered separate from other B cell lymphomas. Field stain. CT scan of primary B cell lymphoma in the left ilium, as diffuse cortical and trabecular thickening of the hemipelvis, mimicking Paget's disease.
B-cells form in the bone marrow and undergo gene rearrangement in order to develop B-cell receptors (BCRs) that bind to a specific antigen. Once activated by an antigen, B-cells proliferate and further differentiate into plasma cells and memory B-cells. [4] B-cells that have not encountered an antigen are called naive B cells.
Four chimeric antigen receptor T cell therapies are FDA-approved for non-Hodgkin lymphoma, including lisocabtagene maraleucel (for relapsed or refractory large B-cell lymphoma with two failed systemic treatments), axicabtagene ciloleucel, tisagenlecleucel (for large B-cell lymphoma), and brexucabtagene autoleucel (for mantle cell lymphoma ...