Progressive TP53 inactivation in an aggressive splenic diffuse red pulp small B-cell lymphoma.

Autor: Tzioni, Maria-Myrsini, Clipson, Alexandra, Chen, Zi, Cucco, Francesco, Wotherspoon, Andrew, Dojcinov, Stefan, Du, Ming-Qing
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Zdroj: Leukemia & Lymphoma; Jul2024, Vol. 65 Issue 7, p1003-1007, 5p
Abstrakt: Splenic diffuse red pulp small B-cell lymphoma (SDRPL) is a rare type of lymphoma that affects the spleen, bone marrow, and peripheral blood. It was initially thought to be a variant of splenic marginal zone lymphoma (SMZL) but is now recognized as a separate entity. SDRPL is difficult to diagnose based on peripheral blood and bone marrow samples alone, and a splenectomy is often necessary for an accurate diagnosis. The pathogenesis of SDRPL is not well understood, but it is associated with specific genetic changes, including mutations in BCOR, CCND3, NOTCH pathway genes, and MAP2K1. The prognosis and treatment response of SDRPL vary, but TP53 inactivation, through mutation and/or deletion, is associated with an aggressive clinical course. [Extracted from the article]
Databáze: Complementary Index