title: Richter Syndrome in Chronic Lymphocytic Leukemia.
abstract: 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.
authors.affiliation: Department of Leukemia, The University of Texas MD Anderson Cancer Center, Unit 428, 1515 Holcombe Boulevard, Houston, TX, 77030, USA.
authors.forename: Candida
authors.initials: C
authors.lastname: Vitale
authors.affiliation: Department of Leukemia, The University of Texas MD Anderson Cancer Center, Unit 428, 1515 Holcombe Boulevard, Houston, TX, 77030, USA. aferrajo@mdanderson.org.
authors.forename: Alessandra
authors.initials: A
authors.lastname: Ferrajoli
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D006801:Humans; D007143:Immunoglobulin Heavy Chains / Q000235:genetics; D015451:Leukemia, Lymphocytic, Chronic, B-Cell / Q000175:diagnosis* / Q000188:drug therapy / Q000401:mortality; D049268:Positron-Emission Tomography; D011379:Prognosis; D047428:Protein Kinase Inhibitors / Q000008:administration & dosage; D012307:Risk Factors; D015996:Survival Rate
chemical_list: D007143:Immunoglobulin Heavy Chains; D047428:Protein Kinase Inhibitors
keywords: B cell lymphoma; Chronic lymphocytic leukemia; Richter syndrome
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