TY - JOUR
T1 - Richter Syndrome in Chronic Lymphocytic Leukemia
AU - Vitale, Candida
AU - Ferrajoli, Alessandra
N1 - Publisher Copyright:
© 2016, Springer Science+Business Media New York.
PY - 2016/2/1
Y1 - 2016/2/1
N2 - The term Richter syndrome (RS) indicates the transformation of chronic lymphocytic leukemia (CLL) into an aggressive lymphoma. RS is a rare complication with an aggressive clinical course, bearing an unfavorable prognosis. In the majority of cases, CLL transforms into RS as diffuse large B cell lymphoma (DLBCL), and a clonal relation between the two processes can be found. However, clonally unrelated RS can occur and transformations to other histologies beside DLBCL have been described. Recent data have shed some light on genetic characteristics that can influence and drive the transformation from CLL to RS. This molecular information has not been translated yet into significant treatment advances, and currently the therapy regimens for RS continue to rely on intensive chemotherapy combinations followed by stem cell transplant in suitable candidates. Based on the rapid pace of discoveries in the field of hematological malignancies and on the recent revolution in the therapeutic landscape for CLL and B cell lymphomas, new therapeutic options for RS might be available in the upcoming years.
AB - The term Richter syndrome (RS) indicates the transformation of chronic lymphocytic leukemia (CLL) into an aggressive lymphoma. RS is a rare complication with an aggressive clinical course, bearing an unfavorable prognosis. In the majority of cases, CLL transforms into RS as diffuse large B cell lymphoma (DLBCL), and a clonal relation between the two processes can be found. However, clonally unrelated RS can occur and transformations to other histologies beside DLBCL have been described. Recent data have shed some light on genetic characteristics that can influence and drive the transformation from CLL to RS. This molecular information has not been translated yet into significant treatment advances, and currently the therapy regimens for RS continue to rely on intensive chemotherapy combinations followed by stem cell transplant in suitable candidates. Based on the rapid pace of discoveries in the field of hematological malignancies and on the recent revolution in the therapeutic landscape for CLL and B cell lymphomas, new therapeutic options for RS might be available in the upcoming years.
KW - B cell lymphoma
KW - Chronic lymphocytic leukemia
KW - Richter syndrome
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U2 - 10.1007/s11899-016-0300-y
DO - 10.1007/s11899-016-0300-y
M3 - Review article
C2 - 26830086
AN - SCOPUS:84961198592
SN - 1558-8211
VL - 11
SP - 43
EP - 51
JO - Current hematologic malignancy reports
JF - Current hematologic malignancy reports
IS - 1
ER -