2009
DOI: 10.1002/hon.880
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Richter syndrome: molecular insights and clinical perspectives

Abstract: Richter syndrome (RS) represents the clinico-pathologic transformation of chronic lymphocytic leukaemia (CLL) to an aggressive lymphoma, most commonly diffuse large B-cell lymphoma (DLBCL). The clinical definition of RS is heterogeneous, and encompasses at least two biologically different conditions: (i) CLL transformation to a clonally related DLBCL, that accounts for the majority of cases; (ii) development of a DLBCL unrelated to the CLL clone. In clonally related RS, the pathogenetic link between the CLL an… Show more

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Cited by 108 publications
(96 citation statements)
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References 80 publications
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“…[1][2][3][4] Despite morphologic and phenotypic similarities, 4 the ID status of IGHV genes of clonally unrelated RS differed from that of both clonally related RS and de novo DLBCL with a non-GC phenotype. In fact, all clonally unrelated RS harbored ID and were characterized by a significantly higher mutation frequency (median: 1.18 Â 10 À3 range: 0.42-1.61 Â 10…”
Section: The Role Of Id Is Different In Clonally Unrelated Rs Versus mentioning
confidence: 99%
See 1 more Smart Citation
“…[1][2][3][4] Despite morphologic and phenotypic similarities, 4 the ID status of IGHV genes of clonally unrelated RS differed from that of both clonally related RS and de novo DLBCL with a non-GC phenotype. In fact, all clonally unrelated RS harbored ID and were characterized by a significantly higher mutation frequency (median: 1.18 Â 10 À3 range: 0.42-1.61 Â 10…”
Section: The Role Of Id Is Different In Clonally Unrelated Rs Versus mentioning
confidence: 99%
“…[1][2][3][4] The role of antigen in promoting CLL transformation to RS is supported by the recent observation of a high prevalence of stereotyped B-cell receptors in CLL transforming to RS, and by the documentation of biased usage of immunoglobulin heavy variable (IGHV) genes, including IGHV4-39.…”
mentioning
confidence: 99%
“…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).…”
Section: Discussionmentioning
confidence: 99%
“…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).…”
Section: Peripheral Blood Lymph Nodementioning
confidence: 99%
“…Among patients with newly diagnosed CLL/SLL, the annual incidence rate of DLBCL is 0.5%; whereas among patients who have received treatment for CLL/SLL the annual incidence rate of DLBCL is 1% [15]. Up to 10.7% of all CLL/SLL patients progress to DLBCL (RS-DLBCL) [11,12,14,15,[20][21][22][23] (Supporting Information Table I). The incidence rate for DLBCL reported in the literature is highly variable, likely attributable to many factors including the heterogeneity of the patient population studied, duration of clinical follow-up, and diagnostic criteria used to define development of DLBCL (clinical/imaging vs. biopsy-proven).…”
Section: Histologic Variants Of Richter Transformationmentioning
confidence: 99%