A systematic review on Richter syndrome: what is the published evidence?
- 18 January 2010
- journal article
- review article
- Published by Informa UK Limited in Leukemia & Lymphoma
- Vol. 51 (3), 415-421
- https://doi.org/10.3109/10428190903515192
Abstract
A systematic and comprehensive search of literature was performed using MEDLINE databases from 1st January 1978 to 2nd November 2009 and hand search of references. A search performed by combining the Medical Subject Headings (MESH) terms 'Richter's syndrome' and 'chronic lymphocytic leukemia' (CLL) yielded 143 citations. Ten additional case-control studies judged relevant for the purpose of study were also included. In total, 45 case reports, 18 case series, and 9 case-control studies were identified. For the purpose of this review, only case series and case-control studies were considered. The following conclusions could be drawn from the studies analyzed in this review: (i) some biological markers (i.e. CD38 expression and genotype, absence of del13q) or clinical features (i.e. bulky lymph node involvement), although not validated in prospective trials, may be considered for close monitoring and a careful biopsy policy; (ii) PET, is not yet standardized in RS, however, it may be useful in the diagnosis and to choose the site for biopsy; (iii) when diagnosed RS should be treated with a combination of rituximab and polychemotherapy; (iii) younger patients who respond to initial therapy should be offered allogeneic SCT, if feasible. Despite the paucity of data, it is important to note that this article represents the first systematic review of the entire body of available clinical evidence useful for an appropriate management of Richter transformation.Keywords
This publication has 28 references indexed in Scilit:
- Other Malignancies in Chronic Lymphocytic Leukemia/Small Lymphocytic LymphomaJournal of Clinical Oncology, 2009
- Biological and clinical risk factors of chronic lymphocytic leukaemia transformation to Richter syndromeBritish Journal of Haematology, 2008
- Guidelines for the diagnosis and treatment of chronic lymphocytic leukemia: a report from the International Workshop on Chronic Lymphocytic Leukemia updating the National Cancer Institute–Working Group 1996 guidelinesBlood, 2008
- Risk factors for development of a second lymphoid malignancy in patients with chronic lymphocytic leukaemiaBritish Journal of Haematology, 2007
- Assessment of fludarabine plus cyclophosphamide for patients with chronic lymphocytic leukaemia (the LRF CLL4 Trial): a randomised controlled trialThe Lancet, 2007
- Risk of second cancer after chronic lymphocytic leukemiaInternational Journal of Cancer, 2007
- Chemoimmunotherapy With Fludarabine, Cyclophosphamide, and Rituximab for Relapsed and Refractory Chronic Lymphocytic LeukemiaJournal of Clinical Oncology, 2005
- Early Results of a Chemoimmunotherapy Regimen of Fludarabine, Cyclophosphamide, and Rituximab As Initial Therapy for Chronic Lymphocytic LeukemiaJournal of Clinical Oncology, 2005
- Richter syndromeCancer, 2005
- Second Malignancies as a Consequence of Nucleoside Analog Therapy for Chronic Lymphoid LeukemiasJournal of Clinical Oncology, 1999