Abstract:
:Richter syndrome (RS) is the development of an aggressive lymphoma in patients with a previous or concomitant diagnosis of chronic lymphocytic leukemia (CLL). The incidence rate RS is ~0.5% per year of observation. Two biomarkers (NOTCH1 mutations and subset 8 configuration of the B-cell receptor) may help identifying CLL patients at risk of RS to be considered for close monitoring and a careful biopsy policy. In the presence of clinical features suspicious of RS, diagnosis of transformation and choice of the site of biopsy may take advantage of fluorine 18 fluorodeoxyglucose ((18)FDG) positron emission tomography (PET)/computed tomography (CT). Molecular lesions of regulators of tumor suppression (TP53), cell cycle (CDKN2A), and cell proliferation (NOTCH1, MYC) overall account for ~90% of RS and may be responsible for the aggressive clinical phenotype observed in this disease because of the combined effect of chemoresistance and rapid disease kinetics. The prognosis of RS is generally highly unfavorable. However, the pattern of survival is not homogeneous and the most important prognostic factor is the clonal relationship between the CLL and the aggressive lymphoma clones. Rituximab-containing polychemotherapy represents the backbone for induction treatment in RS. Younger patients who respond to induction therapy should be offered stem cell transplant (SCT) to prolong survival.
journal_name
Semin Oncoljournal_title
Seminars in oncologyauthors
Rossi D,Gaidano Gdoi
10.1053/j.seminoncol.2016.02.012subject
Has Abstractpub_date
2016-04-01 00:00:00pages
311-9issue
2eissn
0093-7754issn
1532-8708pii
S0093-7754(16)00018-Xjournal_volume
43pub_type
杂志文章,评审abstract::The ability to predict the biological behavior of breast tumors can allow selection of the optimum treatment and follow-up strategies. Conventionally, tumor size, lymph node metastases, and histopathologic features have been used to determine the risk of systemic relapse. The discovery of a number of specific genetic ...
journal_title:Seminars in oncology
pub_type: 杂志文章,评审
doi:
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journal_title:Seminars in oncology
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journal_title:Seminars in oncology
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pub_type: 杂志文章,评审
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journal_title:Seminars in oncology
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journal_title:Seminars in oncology
pub_type: 杂志文章,评审
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journal_title:Seminars in oncology
pub_type: 杂志文章,评审
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journal_title:Seminars in oncology
pub_type: 杂志文章,评审
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journal_title:Seminars in oncology
pub_type: 杂志文章,评审
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journal_title:Seminars in oncology
pub_type: 临床试验,杂志文章,多中心研究
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更新日期:1996-12-01 00:00:00
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journal_title:Seminars in oncology
pub_type: 杂志文章,评审
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journal_title:Seminars in oncology
pub_type: 杂志文章,评审
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更新日期:2001-08-01 00:00:00
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journal_title:Seminars in oncology
pub_type: 临床试验,杂志文章,随机对照试验
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更新日期:2000-06-01 00:00:00
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journal_title:Seminars in oncology
pub_type: 杂志文章,评审
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