How we treat Richter syndrome

Sameer A Parikh, Neil Elliot Kay, Tait D. Shanafelt

Research output: Contribution to journalArticle

77 Citations (Scopus)

Abstract

Richter syndrome (RS) is defined as the transformation of chronic lymphocytic leukemia (CLL) into an aggressive lymphoma, most commonly diffuse large B-cell lymphoma (DLBCL). RS occurs in approximately 2% to 10% of CLL patients during the course of their disease, with a transformation rate of 0.5% to 1% per year. A combination of germline genetic characteristics, clinical features (eg, advanced Rai stage), biologic (δ-associated protein-70+, CD38+, CD49d+) and somatic genetic (del17p13.1 or del11q23.1) characteristics of CLL B cells, and certain CLL therapies areassociatedwith higher risk of RS. Recent studies have also identified the crucial role of CDKN2A loss, TP53 disruption, C-MYC activation, and NOTCH1 mutations in the transformation from CLL to RS. An excisional lymph node biopsy is considered the gold standard for diagnosis of RS; a 18F-fluorodeoxyglucose positron emission tomography scan can help inform the optimal site for biopsy. Approximately 80% of DLBCL cases in patients with CLL are clonally related to the underlying CLL, and the median survival for these patients is approximately 1 year. In contrast, the remaining 20% of patients have a clonally unrelated DLBCL and have a prognosis similar to that of de novo DLBCL. For patients with clonally related DLBCL, induction therapy with either an anthracycline- or platinum-based regimen is the standard approach. Postremission stem cell transplantation should be considered for appropriate patients. This article summarizes our approach to the clinical management of CLL patients who develop RS.

Original languageEnglish (US)
Pages (from-to)1647-1657
Number of pages11
JournalBlood
Volume123
Issue number11
DOIs
StatePublished - Mar 13 2014

Fingerprint

Biopsy
B-Cell Chronic Lymphocytic Leukemia
Cells
Lymphoma, Large B-Cell, Diffuse
Positron emission tomography
Anthracyclines
Fluorodeoxyglucose F18
Platinum
Stem cells
Chemical activation
Proteins
Stem Cell Transplantation
Positron-Emission Tomography
Lymphoma
Lymph Nodes
Mutation
Survival
Therapeutics

ASJC Scopus subject areas

  • Hematology
  • Biochemistry
  • Cell Biology
  • Immunology

Cite this

How we treat Richter syndrome. / Parikh, Sameer A; Kay, Neil Elliot; Shanafelt, Tait D.

In: Blood, Vol. 123, No. 11, 13.03.2014, p. 1647-1657.

Research output: Contribution to journalArticle

Parikh, Sameer A ; Kay, Neil Elliot ; Shanafelt, Tait D. / How we treat Richter syndrome. In: Blood. 2014 ; Vol. 123, No. 11. pp. 1647-1657.
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