Richter syndrome

Richter's and prolymphocytic transformation of chronic lymphocytic leukemia are associated with high mRNA expression of activation-induced cytidine deaminase and aberrant somatic hypermutation

“…Eight cases of CLL (cases [1][2][3][4][5][6][7][8] showed no transformation during the clinical course of the disease, 13 cases (cases 9-21) developed RT and eight cases (cases [22][23][24][25][26][27][28][29] showed PLT. In seven cases of RT (cases 9-15), both CLL and DLBL samples were available for analysis.…”

Successful Allogeneic Hematopoietic Stem Cell Transplantation in a Young Patient with Richter Syndrome Presenting with Chronic Lymphocytic Leukemia and Diffuse Large B-Cell Lymphoma with Different Cell Origins

“…Depending on the patient's age and the RS score, the therapeutic options for treating RS range from conventional immunochemotherapy to alloHSCT. However, intensive immunochemotherapies utilized for high-grade non hodgkin lymphoma (NHL) or (ALL) have not been found to be effective for RS, demonstrating a median survival time of less than one year (3)(4)(5). On the other hand, in a nonrandomized comparison of two RS cohorts, the estimated overall survival rate at three years was 75% among patients who received alloHSCT after achieving a complete remission (CR) or partial remission (PR), compared to 27% among those who responded to the initial therapy but did not receive alloHSCT (6).…”

“…The clinical outcomes of RS are generally poor with a median survival time of less than one year, even when intensive chemotherapies effective for high-grade nonHodgkin's lymphoma or acute lymphoblastic leukemia (ALL) are applied (3)(4)(5). Additionally, as most RS patients are elderly, allogeneic hematopoietic stem cell transplantation (alloHSCT) is applicable in limited cases only (6).…”

Chromosomal abnormalities detected by multicolor fluorescence in situ hybridization in fine-needle aspirates from patients with small lymphocytic lymphoma are useful for predicting survival

“…When trisomy 12 is associated with complex karyotypes, there is an increased risk of transformation to Richter syndrome. 2 Deletion of 13q is the most common genetic abnormality in CLL, occurring in 40% to 65% of cases. The deletion may be limited to a single locus, 13q14, or involve a larger region of chromosome 13.…”

“…1 Approximately 5% of cases will transform to a high-grade lymphoma, clinically referred to as Richter syndrome. 2 At our institution, fine-needle aspiration (FNA) of lymph nodes is a method commonly used to assess disease progression in patients with SLL. Although the cytologic features are helpful in diagnosing typical SLL and transformed large cell lymphoma (tLCL), a subset of cases possibly in transition to tLCL are more difficult to diagnose.…”