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Review
. 2022 Aug;24(8):1081-1090.
doi: 10.1007/s11912-022-01274-4. Epub 2022 Apr 6.

Richter's Transformation

Audrey M Sigmund  1 , Adam S Kittai  2
Affiliations
Review

Richter's Transformation

Audrey M Sigmund et al. Curr Oncol Rep. 2022 Aug.

Abstract

Purpose of review: Richter's transformation (RT) occurs when chronic (CLL) transforms into an aggressive lymphoma. Despite improvements in the treatment of CLL, prognosis for RT remains poor. Here, we review current literature of RT, with a focus on novel treatment options.

Recent findings: Efforts are underway to improve outcomes for patients with RT. While small molecule inhibitors have limited efficacy as monotherapy, recent developments combining them with chemo-immunotherapy show promise. Studies exploring the use of cellular therapies including chimeric antigen receptor T-cells and bispecific antibodies are ongoing. The current treatment paradigm for RT is to enroll these patients on a clinical trial when available, together with consultation for a consolidative allogeneic stem cell transplant. Trials investigating novel combinations and cellular therapy are ongoing. Determining predictive variables of transformation is imperative to design studies that allow for early identification and intervention for patients with RT.

Keywords: Chronic lymphocytic leukemia; Diffuse large B-cell lymphoma; Hodgkin lymphoma; Richter’s transformation; Transformation.

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References

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance
    1. Richter MN. Generalized reticular cell sarcoma of lymph nodes associated with lymphatic leukemia. Am J Pathol. 1928;4(4):285–92 7. - PubMed - PMC
    1. Lortholary P, Boiron M, Ripault P, Levy JP, Manus A, Bernard J. Chronic lymphoid leukemia secondarily associated with a malignant reticulopathy: Richter's syndrome. Nouv Rev Fr Hematol. 1964;4:621–44. - PubMed
    1. Rossi D, Spina V, Gaidano G. Biology and treatment of Richter syndrome. Blood. 2018;131(25):2761–72. - PubMed - DOI
    1. • Wang Y, Tschautscher MA, Rabe KG, Call TG, Leis JF, Kenderian SS, et al. Clinical characteristics and outcomes of Richter transformation: experience of 204 patients from a single center. Haematologica. 2020;105(3):765–73. A large single institution study evaluating clinical characteristics and outcomes in patients with RT. - PubMed - PMC - DOI
    1. • Elnair R, Ellithi M, Kallam A, Shostrom V, Bociek RG. Outcomes of Richter's transformation of chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL): an analysis of the SEER database. Ann Hematol. 2021;100(10):2513–9. A recent analysis utilizing the SEER database evaluating outcomes of patients with RT. - PubMed - DOI

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