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Case 70 - Discussion

Uploaded: 2008-011-22, Updated: 2008-11-22




Diffuse large B-cell lymphoma transformed from CLL (Richterís transformation)

Chronic lymphocytic leukemia (CLL) is the most common type of leukemia worldwide. It is an indolent disease, almost exclusively of B-cell origin. Some CLLs may transform into a more aggressive lymphoid malignancy, with the most common of these is Richter's syndrome. Richter's transformation denotes the development of high-grade non-Hodgkin lymphoma, prolymphocytic leukemia, Hodgkin disease, or acute leukemia in patients with chronic lymphocytic leukemia (CLL)/small lymphocytic lymphoma. Most Richter's syndrome cases are secondary diffuse large B-cell lymphomas, but some are Hodgkin's disease variants. Richter's syndrome occurs in approximately 5% of patients with CLL.

Richter's syndrome may be triggered by viral infections, especially Epstein-Barr virus, and it is also associated with some chromosomal abnormalities and genetic defects, including trisomy 12, chromosome 11 abnormalities, mutations of p53 tumor, p16INK4A, and p21, loss of p27 expression, deletion of retinoblastoma, increased copy number of C-MYC, and decreased expression of the A-MYB gene, expression of activation-induced cytidine deaminase (AID), and aberrant somatic hypermutation (ASHM). The large cells of RS may arise through transformation of the original CLL clone or represent a new neoplasm.



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