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Richter syndrome

MedGen UID:
91159
•Concept ID:
C0349631
•
Neoplastic Process
Synonym: Large cell lymphoma which develops after treatment for chronic lymphocytic leukemia (CLL)
SNOMED CT: Richter syndrome (277550009); Richter's syndrome (277550009)
 
Monarch Initiative: MONDO:0002083

Definition

Transformation of chronic lymphocytic leukemia into aggressive non-Hodgkin lymphoma, usually diffuse large B-cell lymphoma (immunoblastic or centroblastic variant). Occasional cases of transformation to Hodgkin lymphoma have also been described, particularly in patients treated with purine nucleotide analogues. Molecular genetic studies suggest that in approximately half of the cases, the lymphoma is clonally related to the underlying chronic lymphocytic leukemia, whereas in the remaining cases the lymphoma probably represents a secondary, unrelated neoplasm. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVRichter syndrome

Professional guidelines

PubMed

Biology and treatment of Richter syndrome.
Rossi D, Spina V, Gaidano G
Blood 2018 Jun 21;131(25):2761-2772. Epub 2018 Apr 24 doi: 10.1182/blood-2018-01-791376. PMID: 29692342
Treatment of Richter's Syndrome.
Condoluci A, Rossi D
Curr Treat Options Oncol 2017 Nov 21;18(12):75. doi: 10.1007/s11864-017-0512-y. PMID: 29159711
Richter syndrome: pathogenesis and management.
Rossi D, Gaidano G
Semin Oncol 2016 Apr;43(2):311-9. Epub 2016 Feb 15 doi: 10.1053/j.seminoncol.2016.02.012. PMID: 27040710

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) Chronic Lymphocytic Leukemia/ Small Lymphocytic Lymphoma, 2023

Recent clinical studies

Etiology

Richter syndrome: novel insights into the biology of transformation.
Parry EM, Ten Hacken E, Wu CJ
Blood 2023 Jul 6;142(1):11-22. doi: 10.1182/blood.2022016502. PMID: 36758208Free PMC Article
Genomic and transcriptomic correlates of Richter transformation in chronic lymphocytic leukemia.
Klintman J, Appleby N, Stamatopoulos B, Ridout K, Eyre TA, Robbe P, Pascua LL, Knight SJL, Dreau H, Cabes M, Popitsch N, Ehinger M, Martín-Subero JI, Campo E, Månsson R, Rossi D, Taylor JC, Vavoulis DV, Schuh A
Blood 2021 May 20;137(20):2800-2816. doi: 10.1182/blood.2020005650. PMID: 33206936Free PMC Article
Treatment of Richter's Syndrome.
Condoluci A, Rossi D
Curr Treat Options Oncol 2017 Nov 21;18(12):75. doi: 10.1007/s11864-017-0512-y. PMID: 29159711
Richter syndrome: pathogenesis and management.
Rossi D, Gaidano G
Semin Oncol 2016 Apr;43(2):311-9. Epub 2016 Feb 15 doi: 10.1053/j.seminoncol.2016.02.012. PMID: 27040710
Richter syndrome.
Rossi D, Gaidano G
Adv Exp Med Biol 2013;792:173-91. doi: 10.1007/978-1-4614-8051-8_8. PMID: 24014297

Diagnosis

Richter syndrome: novel insights into the biology of transformation.
Parry EM, Ten Hacken E, Wu CJ
Blood 2023 Jul 6;142(1):11-22. doi: 10.1182/blood.2022016502. PMID: 36758208Free PMC Article
Evolutionary history of transformation from chronic lymphocytic leukemia to Richter syndrome.
Parry EM, Leshchiner I, Guièze R, Johnson C, Tausch E, Parikh SA, Lemvigh C, Broséus J, Hergalant S, Messer C, Utro F, Levovitz C, Rhrissorrakrai K, Li L, Rosebrock D, Yin S, Deng S, Slowik K, Jacobs R, Huang T, Li S, Fell G, Redd R, Lin Z, Knisbacher BA, Livitz D, Schneider C, Ruthen N, Elagina L, Taylor-Weiner A, Persaud B, Martinez A, Fernandes SM, Purroy N, Anandappa AJ, Ma J, Hess J, Rassenti LZ, Kipps TJ, Jain N, Wierda W, Cymbalista F, Feugier P, Kay NE, Livak KJ, Danysh BP, Stewart C, Neuberg D, Davids MS, Brown JR, Parida L, Stilgenbauer S, Getz G, Wu CJ
Nat Med 2023 Jan;29(1):158-169. Epub 2023 Jan 9 doi: 10.1038/s41591-022-02113-6. PMID: 36624313Free PMC Article
Treatment of Richter's Syndrome.
Condoluci A, Rossi D
Curr Treat Options Oncol 2017 Nov 21;18(12):75. doi: 10.1007/s11864-017-0512-y. PMID: 29159711
Richter syndrome: pathogenesis and management.
Rossi D, Gaidano G
Semin Oncol 2016 Apr;43(2):311-9. Epub 2016 Feb 15 doi: 10.1053/j.seminoncol.2016.02.012. PMID: 27040710
Richter syndrome.
Rossi D, Gaidano G
Adv Exp Med Biol 2013;792:173-91. doi: 10.1007/978-1-4614-8051-8_8. PMID: 24014297

Therapy

Venetoclax plus dose-adjusted R-EPOCH for Richter syndrome.
Davids MS, Rogers KA, Tyekucheva S, Wang Z, Pazienza S, Renner SK, Montegaard J, Ihuoma U, Lehmberg TZ, Parry EM, Wu CJ, Jacobson CA, Fisher DC, Thompson PA, Brown JR
Blood 2022 Feb 3;139(5):686-689. doi: 10.1182/blood.2021011386. PMID: 34788401Free PMC Article
Richter Syndrome.
Condoluci A, Rossi D
Curr Oncol Rep 2021 Feb 12;23(3):26. doi: 10.1007/s11912-020-01001-x. PMID: 33580422Free PMC Article
ROR1 targeting with the antibody-drug conjugate VLS-101 is effective in Richter syndrome patient-derived xenograft mouse models.
Vaisitti T, Arruga F, Vitale N, Lee TT, Ko M, Chadburn A, Braggio E, Di Napoli A, Iannello A, Allan JN, Miller LL, Lannutti BJ, Furman RR, Jessen KA, Deaglio S
Blood 2021 Jun 17;137(24):3365-3377. doi: 10.1182/blood.2020008404. PMID: 33512452Free PMC Article
Genomic and transcriptomic correlates of Richter transformation in chronic lymphocytic leukemia.
Klintman J, Appleby N, Stamatopoulos B, Ridout K, Eyre TA, Robbe P, Pascua LL, Knight SJL, Dreau H, Cabes M, Popitsch N, Ehinger M, Martín-Subero JI, Campo E, Månsson R, Rossi D, Taylor JC, Vavoulis DV, Schuh A
Blood 2021 May 20;137(20):2800-2816. doi: 10.1182/blood.2020005650. PMID: 33206936Free PMC Article
Treatment of Richter's Syndrome.
Condoluci A, Rossi D
Curr Treat Options Oncol 2017 Nov 21;18(12):75. doi: 10.1007/s11864-017-0512-y. PMID: 29159711

Prognosis

Molecular characterization of Richter syndrome identifies de novo diffuse large B-cell lymphomas with poor prognosis.
Broséus J, Hergalant S, Vogt J, Tausch E, Kreuz M, Mottok A, Schneider C, Dartigeas C, Roos-Weil D, Quinquenel A, Moulin C, Ott G, Blanchet O, Tomowiak C, Lazarian G, Rouyer P, Chteinberg E, Bernhart SH, Tournilhac O, Gauchotte G, Lomazzi S, Chapiro E, Nguyen-Khac F, Chery C, Davi F, Hunault M, Houlgatte R, Rosenwald A, Delmer A, Meyre D, Béné MC, Thieblemont C, Lichter P, Ammerpohl O, Guéant JL; ICGC MMML-Seq Consortium, Guièze R, Martin-Subero JI, Cymbalista F, Feugier P, Siebert R, Stilgenbauer S
Nat Commun 2023 Jan 19;14(1):309. doi: 10.1038/s41467-022-34642-6. PMID: 36658118Free PMC Article
Venetoclax plus dose-adjusted R-EPOCH for Richter syndrome.
Davids MS, Rogers KA, Tyekucheva S, Wang Z, Pazienza S, Renner SK, Montegaard J, Ihuoma U, Lehmberg TZ, Parry EM, Wu CJ, Jacobson CA, Fisher DC, Thompson PA, Brown JR
Blood 2022 Feb 3;139(5):686-689. doi: 10.1182/blood.2021011386. PMID: 34788401Free PMC Article
Richter Syndrome.
Condoluci A, Rossi D
Curr Oncol Rep 2021 Feb 12;23(3):26. doi: 10.1007/s11912-020-01001-x. PMID: 33580422Free PMC Article
Genomic and transcriptomic correlates of Richter transformation in chronic lymphocytic leukemia.
Klintman J, Appleby N, Stamatopoulos B, Ridout K, Eyre TA, Robbe P, Pascua LL, Knight SJL, Dreau H, Cabes M, Popitsch N, Ehinger M, Martín-Subero JI, Campo E, Månsson R, Rossi D, Taylor JC, Vavoulis DV, Schuh A
Blood 2021 May 20;137(20):2800-2816. doi: 10.1182/blood.2020005650. PMID: 33206936Free PMC Article
Richter syndrome.
Rossi D, Gaidano G
Adv Exp Med Biol 2013;792:173-91. doi: 10.1007/978-1-4614-8051-8_8. PMID: 24014297

Clinical prediction guides

Venetoclax plus dose-adjusted R-EPOCH for Richter syndrome.
Davids MS, Rogers KA, Tyekucheva S, Wang Z, Pazienza S, Renner SK, Montegaard J, Ihuoma U, Lehmberg TZ, Parry EM, Wu CJ, Jacobson CA, Fisher DC, Thompson PA, Brown JR
Blood 2022 Feb 3;139(5):686-689. doi: 10.1182/blood.2021011386. PMID: 34788401Free PMC Article
Approach to Richter transformation of chronic lymphocytic leukemia in the era of novel therapies.
Khan M, Siddiqi R, Thompson PA
Ann Hematol 2018 Jan;97(1):1-15. Epub 2017 Oct 23 doi: 10.1007/s00277-017-3149-9. PMID: 29063177
Richter syndrome: pathogenesis and management.
Rossi D, Gaidano G
Semin Oncol 2016 Apr;43(2):311-9. Epub 2016 Feb 15 doi: 10.1053/j.seminoncol.2016.02.012. PMID: 27040710
Richter syndrome.
Rossi D, Gaidano G
Adv Exp Med Biol 2013;792:173-91. doi: 10.1007/978-1-4614-8051-8_8. PMID: 24014297
Richter syndrome: molecular insights and clinical perspectives.
Rossi D, Gaidano G
Hematol Oncol 2009 Mar;27(1):1-10. doi: 10.1002/hon.880. PMID: 19206112

Recent systematic reviews

Allogeneic hematopoietic cell transplantation is an effective treatment for patients with Richter syndrome: A systematic review and meta-analysis.
Aulakh S, Reljic T, Yassine F, Ayala E, Chavez JC, Chanan-Khan A, Pinilla-Ibarz J, Kumar A, Kharfan-Dabaja MA
Hematol Oncol Stem Cell Ther 2021 Mar;14(1):33-40. Epub 2020 May 15 doi: 10.1016/j.hemonc.2020.05.002. PMID: 32473105Free PMC Article
A systematic review on Richter syndrome: what is the published evidence?
Molica S
Leuk Lymphoma 2010 Mar;51(3):415-21. doi: 10.3109/10428190903515192. PMID: 20078326

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      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Curated

    • NCCN, 2023
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) Chronic Lymphocytic Leukemia/ Small Lymphocytic Lymphoma, 2023

    Reviews

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