کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
---|---|---|---|---|
2161756 | 1091206 | 2016 | 9 صفحه PDF | دانلود رایگان |
عنوان انگلیسی مقاله ISI
Richter syndrome: pathogenesis and management
ترجمه فارسی عنوان
سندرم ریشتر: پاتوژنز و مدیریت
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کلمات کلیدی
موضوعات مرتبط
علوم زیستی و بیوفناوری
بیوشیمی، ژنتیک و زیست شناسی مولکولی
تحقیقات سرطان
چکیده انگلیسی
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 (18FDG) 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.
ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Seminars in Oncology - Volume 43, Issue 2, April 2016, Pages 311-319
Journal: Seminars in Oncology - Volume 43, Issue 2, April 2016, Pages 311-319
نویسندگان
Davide Rossi, Gianluca Gaidano,