Richter Syndrome

被引：19

作者：

Condoluci, Adalgisa ^{[1
,2
]}

Rossi, Davide ^{[1
,2
]}

机构：

[1] Oncol Inst Southern Switzerland, Div Hematol, Bellinzona, Switzerland

[2] Inst Oncol Res, Lab Expt Hematol, Bellinzona, Switzerland

来源：

CURRENT ONCOLOGY REPORTS | 2021年 / 23卷 / 03期

关键词：

Richter syndrome; CLL; Transformation; DLBCL; Hodgkin lymphoma; CHRONIC-LYMPHOCYTIC-LEUKEMIA; IBRITUMOMAB TIUXETAN RADIOIMMUNOTHERAPY; B-CELL LYMPHOMA; PHASE I-II; FRACTIONATED CYCLOPHOSPHAMIDE; HODGKIN TRANSFORMATION; LIPOSOMAL DAUNORUBICIN; RETROSPECTIVE ANALYSIS; CLINICAL-TRIAL; HIGH-RISK;

D O I：

10.1007/s11912-020-01001-x

中图分类号：

R73 [肿瘤学];

学科分类号：

100214 ;

摘要：

Purpose of ReviewRichter syndrome (RS) is an uncommon but aggressive evolution of chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL). RS is an unmet clinical need in the field of CLL. Recent advances in understanding the biology of this condition provide the rationale for testing new therapeutic concepts in order to improve the outcome of patients developing RS, which is so far poor. In this review, we summarize disease characteristics and available therapeutic options for RS.Recent FindingsCurrent regimens with novel agents in monotherapy have shown little impact on survival. Nevertheless, the better reported outcome for RS has been achieved with the combination of chemo-immunotherapy with a novel agent, confirming the synergistic effect of the approaches. Still, the frailty of this population may impose a less toxic management leaving most patients with no reasonable therapeutic option.SummaryTreatment options for RS need to be further expanded. Preclinical models in current development may allow to explore actionable pathways and identify new drug targeted combinations.

引用

页数：10

共 73 条

[11] Targeting BTK with Ibrutinib in Relapsed Chronic Lymphocytic Leukemia [J].

Byrd, John C. ;

Furman, Richard R. ;

Coutre, Steven E. ;

Flinn, Ian W. ;

Burger, Jan A. ;

Blum, Kristie A. ;

Grant, Barbara ;

Sharman, Jeff P. ;

Coleman, Morton ;

Wierda, William G. ;

Jones, Jeffrey A. ;

Zhao, Weiqiang ;

Heerema, Nyla A. ;

Johnson, Amy J. ;

Sukbuntherng, Juthamas ;

Chang, Betty Y. ;

Clow, Fong ;

Hedrick, Eric ;

Buggy, Joseph J. ;

James, Danelle F. ;

O'Brien, Susan .

NEW ENGLAND JOURNAL OF MEDICINE, 2013, 369 (01) :32-42

[12] Two main genetic pathways lead to the transformation of chronic lymphocytic leukemia to Richter syndrome [J].

Chigrinova, Ekaterina ;

Rinaldi, Andrea ;

Kwee, Ivo ;

Rossi, Davide ;

Rancoita, Paola M. V. ;

Strefford, Jonathan C. ;

Oscier, David ;

Stamatopoulos, Kostas ;

Papadaki, Theodora ;

Berger, Francoise ;

Young, Ken H. ;

Murray, Fiona ;

Rosenquist, Richard ;

Greiner, Timothy C. ;

Chan, Wing C. ;

Orlandi, Ester M. ;

Lucioni, Marco ;

Marasca, Roberto ;

Inghirami, Giorgio ;

Ladetto, Marco ;

Forconi, Francesco ;

Cogliatti, Sergio ;

Votavova, Hana ;

Swerdlow, Steven H. ;

Stilgenbauer, Stephan ;

Piris, Miguel A. ;

Matolcsy, Andras ;

Spagnolo, Dominic ;

Nikitin, Eugene ;

Zamo, Alberto ;

Gattei, Valter ;

Bhagat, Govind ;

Ott, German ;

Zucca, Emanuele ;

Gaidano, Gianluca ;

Bertoni, Francesco .

BLOOD, 2013, 122 (15) :2673-2682

[13] Autologous and Allogeneic Stem-Cell Transplantation for Transformed Chronic Lymphocytic Leukemia (Richter's Syndrome): A Retrospective Analysis From the Chronic Lymphocytic Leukemia Subcommittee of the Chronic Leukemia Working Party and Lymphoma Working Party of the European Group for Blood and Marrow Transplantation [J].

Cwynarski, Kate ;

van Biezen, Anja ;

de Wreede, Liesbeth ;

Stilgenbauer, Stephan ;

Bunjes, Donald ;

Metzner, Bernd ;

Koza, Vladimir ;

Mohty, Mohamad ;

Remes, Kari ;

Russell, Nigel ;

Nagler, Arnon ;

Scholten, Marijke ;

de Witte, Theo ;

Sureda, Anna ;

Dreger, Peter .

JOURNAL OF CLINICAL ONCOLOGY, 2012, 30 (18) :2211-2217

[14] Fractionated cyclophosphamide, vincristine, liposomal daunorubicin (daunoxome), and dexamethasone (hyperCVXD) regimen in Richter's syndrome [J].

Dabaja, BS ;

O'Brien, SM ;

Kantarjian, HM ;

Cortes, JE ;

Thomas, DA ;

Albitar, M ;

Schlette, ES ;

Faderl, S ;

Sarris, A ;

Keating, MJ ;

Giles, FJ .

LEUKEMIA & LYMPHOMA, 2001, 42 (03) :329-337

[15] Phase I First-in-Human Study of Venetoclax in Patients With Relapsed or Refractory Non-Hodgkin Lymphoma [J].

Davids, Matthew S. ;

Roberts, Andrew W. ;

Seymour, John F. ;

Pagel, John M. ;

Kahl, Brad S. ;

Wierda, William G. ;

Puvvada, Soham ;

Kipps, Thomas J. ;

Anderson, Mary Ann ;

Salem, Ahmed Hamed ;

Dunbar, Martin ;

Zhu, Ming ;

Peale, Franklin ;

Ross, Jeremy A. ;

Gressick, Lori ;

Desai, Monali ;

Kim, Su Young ;

Verdugo, Maria ;

Humerickhouse, Rod A. ;

Gordon, Gary B. ;

Gerecitano, John F. .

JOURNAL OF CLINICAL ONCOLOGY, 2017, 35 (08) :826-833

[16]

Davids MS, 2020, J CLIN ONCOL, V38

[17] Richter's syndrome (RS) in patients with chronic lymphocytic leukemia (CLL) on novel agent therapy. [J].

Davids, Matthew Steven ;

Huang, Ying ;

Rogers, Kerry Anne ;

Stern, Robert ;

Brown, Jennifer R. ;

Thompson, Philip A. ;

Brander, Danielle M. ;

Danilov, Alexey Valeryevich ;

Ujjani, Chaitra Shankar ;

Parikh, Sameer Ashok ;

Barrientos, Jacqueline Claudia ;

Tam, Constantine ;

Woyach, Jennifer Ann .

JOURNAL OF CLINICAL ONCOLOGY, 2017, 35

[18] MGA, a suppressor of MYC, is recurrently inactivated in high risk chronic lymphocytic leukemia [J].

De Paoli, Lorenzo ;

Cerri, Michaela ;

Monti, Sara ;

Rasi, Silvia ;

Spina, Valeria ;

Bruscaggin, Alessio ;

Greco, Mariangela ;

Ciardullo, Carmela ;

Fama, Rosella ;

Cresta, Stefania ;

Maffei, Rossana ;

Ladetto, Marco ;

Martini, Maurizio ;

Laurenti, Luca ;

Forconi, Francesco ;

Marasca, Roberto ;

Larocca, Luigi M. ;

Bertoni, Francesco ;

Gaidano, Gianluca ;

Rossi, Davide .

LEUKEMIA & LYMPHOMA, 2013, 54 (05) :1087-1090

[19] Pembrolizumab in patients with CLL and Richter transformation or with relapsed CLL [J].

Ding, Wei ;

LaPlant, Betsy R. ;

Call, Timothy G. ;

Parikh, Sameer A. ;

Leis, Jose F. ;

He, Rong ;

Shanafelt, Tait D. ;

Sinha, Sutapa ;

Le-Rademacher, Jennifer ;

Feldman, Andrew L. ;

Habermann, Thomas M. ;

Witzig, Thomas E. ;

Wiseman, Gregory A. ;

Lin, Yi ;

Asmus, Erik ;

Nowakowski, Grzegorz S. ;

Conte, Michael J. ;

Bowen, Deborah A. ;

Aitken, Casey N. ;

Van Dyke, Daniel L. ;

Greipp, Patricia T. ;

Liu, Xin ;

Wu, Xiaosheng ;

Zhang, Henan ;

Secreto, Charla R. ;

Tian, Shulan ;

Braggio, Esteban ;

Wellik, Linda E. ;

Micallef, Ivana ;

Viswanatha, David S. ;

Yan, Huihuang ;

Chanan-Khan, Asher A. ;

Kay, Neil E. ;

Dong, Haidong ;

Ansell, Stephen M. .

BLOOD, 2017, 129 (26) :3419-3427

[20] Evolution to Plasmablastic Lymphoma (PBL) after CAR-T Cell Therapy in a Case of SLL/CLL with Richter's Transformation [J].

Evans, Andrew ;

Burack, Richard ;

Rothberg, Paul G. ;

Porter, David ;

Liesveld, Jane L. .

BLOOD, 2014, 124 (21)

← 1 2 3 4 5 6 7 8 →