Richter syndrome epidemiology in a large population based chronic lymphocytic leukemia cohort from Norway

2019 ◽  
Vol 60 ◽  
pp. 128-133 ◽  
Author(s):  
Andrea Lenartova ◽  
Ulla Randen ◽  
Tom Børge Johannesen ◽  
Geir Erland Tjønnfjord
Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 2852-2852
Author(s):  
Marek Trneny ◽  
Petra Obrtlikova ◽  
Jiri Schwarz ◽  
Tomas Pavlik ◽  
Jan Muzik ◽  
...  

Abstract Abstract 2852 Background: The chronic lymphocytic leukemia is the most frequent leukemia in the western world predominantly diagnosed in older population. It is still considered to be incurable disease, but the significant proportion of patients do not require any treatment during the course of disease. The outcome is influenced by many factors including leukemia biology, patient's status, complications based on immune dysfunction, treatment choice, supportive care and other factors. Clinical trials are focused on highly selected population in need of treatment. Population based data providing the full picture is extremely rare, especially with the treatment data. The presented population-based study provides the full picture of CLL population including the untreated as well treated cohorts in different age and gender subgroups. Methods: Using data from the nationwide, population-based Czech Cancer Registry (CCR) and Czech population life-tables provided by the Czech Statistical Office to the Human Mortality Database stratified by age, sex, and calendar time we characterized trends in incidence, mortality and prevalence for all pts diagnosed with CLL in Czech Republic 1979–2008 and relative survival for patients diagnosed in five calendar periods (1980–1984, 1985–1989, 1990–1994, 1995–1999, 2000–2003). All computations were performed using Stata 10.1™ software. Results: Altogether 13, 162 pts with CLL diagnosis have been reported to the CCR from 1979 till 2008. Median age 70 years at diagnosis remains unchanged during the whole period, the male/female ratio was 1.5: 1. The CLL incidence increased from 3.3 in 1979 to 5.6 per 100 thousands inhabitants in 2008. The therapy was administered in 54% of all patients, with the significant trend to decrease with calendar period from 60.4% for pts dg in 1980–84 period to 42.3% in 200019403 period. The treatment was administered in 70.2% of pts younger than 60 y compared to 50.1% of older than 60y with the trend to decreased number of treated pts during the time in both cohorts (from 78.5% to 55.3% for younger and from 55.4% to 38.7% for older cohort). The improvement in five years relative survival ratios (RSRs; 95% confidence interval) with calendar period was observed for all patients starting at 0.46 (0.39–0.53), 0.48 (0.42–0.55), 0.52 (0.46–0.59), 0.60 (0.54–0.66) to 0.69 (0.62–0.74). The 5 y RSRs was better for untreated patients compared to treated pts resp. with improvement in both cohorts from 0.53 (0.44–0–62) and 0.34 (0.25–0.45) resp. in 1980–4 period to 0.77 (0.70–0.84) and 0.51 (0.42–0.60) resp. in 2000–3 period. The same trend was observed for young and old, untreated and treated pts. The females have significant better outcome compared to males in all cohorts, young as well old, untreated as well treated. Conclusions: In this large population-based study with more than 13, 000 patients we confirmed the increased CLL incidence together with decreased need of treatment which can be explained by the higher number of patients diagnosed in early stages in the recent time. The patients without need of treatment have siginificantly better 5y RSRs compared to pts with need of treatment, the females have significantly better 5y RSRs compared to males consistently in all subgroups. The patient's survival was improved with calendar period for the whole CLL population as well for the younger and older, females and males, untreated as well treated cohort. Disclosures: No relevant conflicts of interest to declare.


2000 ◽  
Vol 124 (6) ◽  
pp. 907-909 ◽  
Author(s):  
Maureen J. O'Sullivan ◽  
Zahid Kaleem ◽  
Michael J. Bolger ◽  
Paul E. Swanson ◽  
Mary M. Zutter

Abstract The indolent course of chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) is occasionally altered by transformation to a histologically distinct, rapidly progressive, and clinically unresponsive hematologic malignant neoplasm. We report a case of CLL that, after 3 years of slowly progressive disease and treatment with single-agent chemotherapy (fludarabine phosphate), underwent a composite prolymphocytoid and classic Hodgkin lymphoma transformation. The diagnosis of classic Hodgkin lymphoma was based on the presence of Reed-Sternberg cells with typical morphologic structure and immunophenotype (CD15+, CD30+, CD45−, CD20−) associated with the characteristic polymorphous inflammatory background consisting of numerous eosinophils, plasma cells, and reactive T lymphocytes. The remainder of the lymph node and the peripheral blood showed increased numbers of prolymphocytes admixed with typical small CLL cells. Recognition of such a transformation is of the utmost importance, since histologically similar Reed-Sternberg–like cells may be seen in Richter transformation. In contrast to prolymphocytoid transformation of CLL, Richter syndrome is rapidly fatal, with a median survival of 4 to 5 months. The patient pursued a clinical course similar to pure prolymphocytoid transformation and died with disease after 30 months following treatment with combination chemotherapy.


2017 ◽  
Vol 103 (1_suppl) ◽  
pp. S37-S40 ◽  
Author(s):  
Elisa Albi ◽  
Stefano Baldoni ◽  
Patrizia Aureli ◽  
Erica Dorillo ◽  
Beatrice Del Papa ◽  
...  

Purpose Richter syndrome (RS) is a rare event in chronic lymphocytic leukemia (CLL) that is influenced by biological factors and prior CLL treatments. Ibrutinib is a Bruton tyrosine kinase inhibitor that has shown remarkable efficacy in CLL; however, little is known about its relationship to RS. We report a case of ibrutinib efficacy against CLL in a patient with prolonged remission of RS. Methods The patient was diagnosed with CLL in 2003. Biological findings at onset included absent ZAP70 expression, mutated IGVH, and NOTCH1 mutation. He was treated with FCR with partial response. In 2013, he progressed to RS, not clonally related to the underlying CLL. The patient was treated with anthracycline- and platinum-based regimens, obtaining a complete remission. After 3 years, he presented a CLL progression with worsening lymphocytosis, anemia, thrombocytopenia, increased splenomegaly, and lymphadenopathies. Positron emission tomography-computed tomography scan excluded pathologic uptake. Thus, he was started on ibrutinib. Results At 12 months’ follow-up, we observed white blood cell normalization, increased hemoglobin and platelet levels, disappearance of lymphadenopathy, and spleen size reduction. Therapy was well-tolerated with no evidence of RS. Conclusion This case demonstrates sustained RS remission in a patient with CLL under ibrutinib therapy, thus improving our knowledge on the use of this new drug in CLL and beyond.


PLoS ONE ◽  
2018 ◽  
Vol 13 (12) ◽  
pp. e0208180 ◽  
Author(s):  
Caroline Holm Nørgaard ◽  
Nikoline Buus Søgaard ◽  
Jorne Lionel Biccler ◽  
Laura Pilgaard ◽  
Mathias Holmsgaard Eskesen ◽  
...  

Cancers ◽  
2020 ◽  
Vol 12 (7) ◽  
pp. 1773 ◽  
Author(s):  
Marika Porrazzo ◽  
Emanuele Nicolai ◽  
Mara Riminucci ◽  
Candida Vitale ◽  
Marta Coscia ◽  
...  

The role of positron emission tomography/computed tomography (PET/CT) in identifying Richter Syndrome (RS) is well established, while its impact on the survival of patients with chronic lymphocytic leukemia (CLL) has been less explored. The clinical characteristics and PET/CT data of 40 patients with a biopsy-proven CLL who required frontline chemoimmunotherapy, FCR (fludarabine, cyclophosphamide, rituximab) in 20 patients, BR (bendamustine, rituximab) in 20, were retrospectively analyzed. Standardized uptake volume (SUVmax) values ≥ 5 were observed more frequently in patients with deletion 11q (p = 0.006) and biopsies characterized by a rate of Ki67 positive cells ≥ 30% (p = 0.02). In the multivariate analysis, the presence of large and confluent PCs emerged as the only factor with a negative impact on progression-free survival (PFS), and overall survival (OS). Deletion 11q also revealed a significant and independent effect on PFS. SUVmax values ≥ 5 showed no statistical impact on PFS while in multivariate analysis, they revealed a significant adverse impact on OS (median survival probability not reached vs. 56 months; p = 0.002). Moreover, patients with higher SUVmax values more frequently developed Richter Syndrome (p = 0.015). Our results show that higher SUVmax values identify CLL patients with a pronounced rate of proliferating cells in the lymph-node compartment, inferior survival, and an increased risk of developing RS.


2011 ◽  
Vol 53 (5) ◽  
pp. 993-995 ◽  
Author(s):  
Michael Mian ◽  
Silvia Cerú ◽  
Atto Billio ◽  
Carlo Rosanelli ◽  
Sergio Cortelazzo

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