Clinical characteristics and outcomes of Richter transformation: experience of 204 patients from a single center

Wang, Yucai; Tschautscher, Marcella; Rabe, Kari G.; Call, Timothy G.; Leis, José F.; Kenderian, Saad S.; Kay, Neil E.; Muchtar, Eli; Dyke, Daniel L. Van; Koehler, Amber B.; Schwager, Susan M.; Slager, Susan L.; Parikh, Sameer A.; Ding, Wei

doi:10.3324/haematol.2019.224121

Cited by 91 publications

(114 citation statements)

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“…In the present large population-based cohort of patients with RS obtained from GELLC centres over three decades, the median OS was only 8 months, similar to that previously reported in other series of patients with RS. 8,12,13 Even though in our present series OS improved over time, particularly since the introduction of rituximab, the clinical A platelet count of <100 9 10 9 /l, prior CLL therapy and TP53 alterations in the CLL clone were the variables most strongly associated with OS in the present series, maintaining an independent prognostic impact in the multivariate analysis. Considering these three variables, we were able to identify a subgroup of patients with DLBCL-type RS (17Á5%) with a better clinical outcome (median OS of 75Á3 months).…”

Section: Discussionmentioning

confidence: 45%

“…13 TP53 disruptions in patients with RS have been recurrently found to be associated with a worse outcome. 8,[13][14][15]31 Regrettably, data about TP53 mutations in the RS biopsies were not available for the majority of cases in our present series. Nevertheless, data on genetic ABVD: adriamycin, bleomycin, vinblastine, dacarbazine; CHOP: cyclophosphamide, doxorubicin, vincristine, prednisone; CMOPP: cyclophosphamide, procarbazine, vincristine, prednisone; VEPEMB: vinblastine, cyclophosphamide, procarbazine, etoposide, mitoxantrone and bleomycin.…”

Section: Discussionmentioning

confidence: 99%

“…8,10,11 Owing to the profile of genetic abnormalities, the underlying CLL, and the aggressiveness of the disease, DLBCL-type RS is associated with a dismal prognosis with a median survival of <1 year. 8,12,13 Different prognostic factors have been described, including the detection of alterations in TP53, the presence of a clonal relationship between CLL and RS, and the number of lines of treatment prior to the diagnosis of RS. 8,12,13 In addition, two different prognostic score indexes have been developed.…”

mentioning

confidence: 99%

“…8,12,13 Different prognostic factors have been described, including the detection of alterations in TP53, the presence of a clonal relationship between CLL and RS, and the number of lines of treatment prior to the diagnosis of RS. 8,12,13 In addition, two different prognostic score indexes have been developed. 8,12 Treatment of patients with DLBCL-type RS is usually based on regimens commonly used in patients with de novo DLBCL, particularly R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone)-like therapies, with discouraging results.…”

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confidence: 99%

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Clinical outcome and prognostic factors of patients with Richter syndrome: real‐world study of the Spanish Chronic Lymphocytic Leukemia Study Group (GELLC)

et al. 2020

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Summary Richter syndrome (RS) is an uncommon evolution of chronic lymphocytic leukaemia (CLL) with a dismal prognosis. Clinical‐biological features predicting outcome and best therapeutic approach for these patients remain to be established. In this study, 128 patients with RS, including 112 diffuse large B‐cell lymphoma (DLBCL)‐type RS, 15 Hodgkin lymphoma (HL)‐type RS, and one plasmablastic lymphoma, were identified in 11 centres of the Spanish CLL Study Group (GELLC). The median overall survival (OS) was 5·9 months for DLBCL‐type RS and 30·8 months for HL‐type RS. Eastern Cooperative Oncology Group Performance Status, haemoglobin level, platelet count, serum lactate dehydrogenase and β2‐microglobulin levels, tumour protein p53 (TP53) abnormalities in the CLL clone concomitant to RS, number of prior therapies, and clonal relationship between CLL and RS, were associated with OS in patients with DLBCL‐type RS. A platelet count of <100 × 109/l, prior CLL therapy (0 vs. ≥1), and presence of TP53 alterations maintained an independent prognostic impact in the multivariate analysis. Patients without any of these factors had a better clinical outcome, with a median OS of 75·3 months, while patients with one or two or more of these factors presented a median OS of 25·5 and 3 months, respectively. Although OS of patients with RS is generally poor, a proportion of patients achieved prolonged survival. Treatment of RS remains a medical need, and further therapeutic approaches with novel therapies are warranted.

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Section: Discussionmentioning

confidence: 45%

Section: Discussionmentioning

confidence: 99%

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confidence: 99%

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confidence: 99%

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Clinical outcome and prognostic factors of patients with Richter syndrome: real‐world study of the Spanish Chronic Lymphocytic Leukemia Study Group (GELLC)

et al. 2020

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“…Recently, Wang and colleagues from the Mayo clinic published their experience of 204 RS patients across 25 years 9 . In the largest RS series presented in the literature to date, they found patients treated with immunochemotherapy who had received no prior treatment for CLL had significantly better overall survival than those pretreated (median 46·3 vs. 7·8 months; P < 0·001).…”

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confidence: 99%

Prognostication in Richter syndrome: context is everything

Eyre

2020

Br J Haematol

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Richter syndrome (RS) represents an aggressive transformation, most typically to diffuse large B-cell lymphoma (DLBCL), from chronic lymphocytic leukaemia (CLL). 1,2 The phenomenon is relatively uncommon, documented in approximately 3% of pre-treated patients with CLL. 3 Standard treatment involves combination immunochemotherapy, which is most commonly anthracycline-based alongside an anti-CD20 monoclonal antibody. 4,5 The prognostic impact of clinical and biological factors associated with patient outcome in RS is still relatively poorly understood, owing in part to the rarity of the entity, and the relative lack of large, high-quality data sets in the modern therapeutic era. Historical series have shown that RS patients have a median overall survival of approximately eight months, with prognostic scoring systems highlighting adverse factors such as thrombocytopenia, poor Eastern Cooperative Oncology Group (ECOG) performance status, raised serum lactate dehydrogenase (LDH), tumour bulk and number of prior therapeutic lines influencing survival outcome. 6,7 Abrisqueta and colleagues from the Spanish CLL Study Group (GELLC) have published in this edition of the British Journal of Haematology outcomes of 112 DLBCL-type RS collated over 30 years across 11 academic sites. 8 Their data have shown that the median overall survival of DLBCL-type RS patients was only 5Á9 months. Importantly, prior therapeutic treatment lines (0 vs. ≥1) [hazard ratio (HR) = 3Á30 (95% CI: 1Á32-8Á19), P = 0Á01], 17p deletion and/or TP53 mutation status [HR = 3Á61 (95% CI: 1Á58-8Á22), P < 0Á01] and platelet count <100 9 10 9 /l [HR = 3Á13 (95% CI: 1Á36-7Á2), P < 0Á01] were clear independent prognostic factors associated with overall survival in their series. Of particular

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Patient characteristics that predict Richter's transformation in patients with chronic lymphocytic leukemia treated with ibrutinib

et al. 2022

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Chronic lymphocytic leukemia (CLL) transformation to aggressive lymphoma, known as Richter's Transformation (RT), has a dismal prognosis. There are limited data evaluating risk of RT in patients treated with ibrutinib. We performed a retrospective analysis to determine prognostic variables associated with development of RT and overall survival (OS) at progression after treatment with ibrutinib. We identified 559 patients with CLL treated with ibrutinib from 2010–2019. After a median follow‐up of 44.5 months from ibrutinib start, 179 patients progressed and were included in our analysis. After a median follow‐up of 20.8 months from progression, 54 out of 179 patients developed RT. Progression on treatment (hazard ratio [HR] 4.01 [1.60–10.00], p = .003), higher LDH (HR 1.80 for 2‐fold increase [1.33–2.43], p = .0001), and lymphadenopathy without lymphocytosis (HR 2.88 [1.15–7.20], p = .02) were independent prognostic variables for the development of RT at progression. Progression with lymphadenopathy without lymphocytosis continued to be an independent prognostic variable of worse OS post‐progression. In a subset analysis of 50 patients who obtained a PET‐CT at progression, the median SUVmax for patients who would develop RT was 15.2 (n = 30, range: 4.0–46.3) versus those patients who did not develop RT with a SUVmax of 7.7 (n = 20, range: 2.3–27.2) (p = .0030). Median OS from date of RT was 4.0 months, suggesting that prognosis for RT remains poor. A lymph node biopsy to rule out RT should be considered in patients who received ibrutinib who progress on treatment, have an elevated LDH, or progress with lymphadenopathy without lymphocytosis.

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Clinical characteristics and outcomes of Richter transformation: experience of 204 patients from a single center

Cited by 91 publications

References 0 publications

Clinical outcome and prognostic factors of patients with Richter syndrome: real‐world study of the Spanish Chronic Lymphocytic Leukemia Study Group (GELLC)

Clinical outcome and prognostic factors of patients with Richter syndrome: real‐world study of the Spanish Chronic Lymphocytic Leukemia Study Group (GELLC)

Prognostication in Richter syndrome: context is everything

Patient characteristics that predict Richter's transformation in patients with chronic lymphocytic leukemia treated with ibrutinib

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