Richter’s transformation in chronic lymphocytic leukemia

Department of Leukemia, The University of Texas M.D. Anderson Cancer Center, 1515 Holcombe Boulevard, Unit 428, Houston, TX 77030, USA.
Current Hematologic Malignancy Reports (Impact Factor: 2.2). 10/2007; 2(4):265-71. DOI: 10.1007/s11899-007-0036-9
Source: PubMed


Richter's syndrome (RS) is the development of high-grade non-Hodgkin's lymphoma (NHL) in patients with chronic lymphocytic leukemia (CLL) or small lymphocytic lymphoma. This process may be triggered by viral infections (eg, Epstein-Barr virus infection), which are common in immunosuppressed patients. The large cells of RS either arise through a transformation of the original CLL clone or, less frequently, represent a new or secondary neoplasm. Karyotypic changes, including trisomy 12, chromosome 11 abnormalities, and multiple cell-cycle regulator disruptions, have been found in patients with RS. Although these genetic defects are believed to cause CLL cells to proliferate and, by facilitating the acquisition of new genetic abnormalities, to transform into RS cells, none appears predominantly responsible for the transformation. The prognosis is generally poor, and most patients do not have long-term (durable) responses to therapy. Rituximab and cytotoxic combination therapy followed by stem cell transplantation is associated with improved clinical outcome. Curative treatment strategies are needed.

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    • "CLL is a heterogeneous disease with variable prognosis; some patients have an indolent course, while others have aggressive disease and a short survival (1,2). Development of high-grade non-Hodgkin’s lymphoma (NHL) in patients with CLL or SLL is termed as Richter’s transformation (RT) (3). Most commonly, the histology seen in patients with RT is the diffuse large B-cell lymphoma (DLBCL), prolymphocytic leukemia as well as Hodgkin lymphoma (HL) and T-cell lymphoma. "
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    ABSTRACT: Richter's transformation (RT) represents the development of high grade lymphoma, most commonly diffuse large B-cell lymphoma, in patients with chronic lymphocytic leukemia or small lymphocytic lymphoma (CLL/SLL). CLL/SLL may convert also to Hodgkin's lymphoma, the so-called Hodgkin's variant of Richter transformation. Histopathological proof is needed to confirm a definitive diagnosis. Patients with RT generally have a poor prognosis, with prompt recognition optimise clinical management. Whole-body PET scan with 18F-FDG can be used for detection of RT of CLL/SLL. We describe the case of 64-year-old woman with CLL/SLL who developed Hodgkin lymphoma detected with PET/CT.
    Full-text · Article · Jun 2014
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    • "lymphoblastic leukemia (CLL) and Hodgkins lymphoma were found to be positive for Epstein-Barr virus (EBV) by polymerase chain reaction (Matsuoka and Jeang, 2007; Montserrat, 2005; Tsimberidou et al., 2007). These results are suggestive that EBV, a B-lymphotropic human herpes virus and may be other viruses may play a role in the progression of some types of leukemia (Rolston and Bodey, 2010; Hasserjian, 2011). "
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    ABSTRACT: ARTICLE INFO ABSTRACT Viruses, also known as oncoviruses, capable of transforming cells into a malignant phenotype. These viruses encode oncogenes that can be inserted into the mammalian genome causing transformation. This study was aimed to detect three types of herpes viruses HHV 4, HHV 6 and HHV 8 sequences in leukemia patients and healthy individuals to evaluate the role of these viruses in leukemia arise, and to determine the best way to detect the virus particles in human sera and blood. The present work were obtained four types of leukemia, Acute Lymphoid Leukemia (ALL), Acute Myeloid Leukemia (AML), Chronic Lymphoid Leukemia (CLL) and Chronic Myeloid Leukemia (CML). The Samples of blood were taken from 75 Iraqi patients newly diagnosed affected with one of the four types of leukemia aged between (2-63) years, and twenty-five healthy control subjects ages between (8-62) year were analysis by molecular genetic methods. During the period from August 2012 till May 2013. The viral DNA extraction from serum by QI Aamp Ultra Sens Virus Kit from Qiageen/Germany was found more suitable and sensitive to detect the present viruses than the genomic DNA extraction from whole blood, further amplified by Polymerase Chain Reaction (PCR). PCR products were analyzed by electrophoresis on 2% agarose gels. The present study detected EBV serologically in serum of patients and controls by the MONO mononucleosis rapid test. The results revealed the molecular way was more effective and sensitive that EBV detect in serology in 2.7% but with molecular methods detect in 19% of samples. These percentage of EBV divided on the four types of leukemia in different numbers, 12% in ALL, 3% in AML, 4% in CLL, where there was no positive samples to HHV-6 and HHV-8. Copyright © Abdul Hussein Moyet Al Faisal and Noor Isam Al Baiyati. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
    Full-text · Article · Oct 2013
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    • "It is thought to be preceded by monoclonal B-cell lymphocytosis (MBL), a state in which a smaller size B-cell clone is present, typically in the absence of symptoms [3]. At the other end of the spectrum, CLL may transform into a higher-grade malignancy, a process termed Richter's transformation, which is often associated with a dismal clinical outcome [4]. "
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    ABSTRACT: Chronic lymphocytic leukemia (CLL) has been consistently at the forefront of genetic research owing to its prevalence and the accessibility of sample material. Recently, genome-wide technologies have been intensively applied to CLL genetics, with remarkable progress. Single nucleotide polymorphism arrays have identified recurring chromosomal aberrations, thereby focusing functional studies on discrete genomic lesions and leading to the first implication of somatic microRNA disruption in cancer. Next-generation sequencing (NGS) has further transformed our understanding of CLL by identifying novel recurrently mutated putative drivers, including the unexpected discovery of somatic mutations affecting spliceosome function. NGS has further enabled in-depth examination of the transcriptional and epigenetic changes in CLL that accompany genetic lesions, and has shed light on how different driver events appear at different stages of disease progression and clonally evolve with relapsed disease. In addition to providing important insights into disease biology, these discoveries have significant translational potential. They enhance prognosis by highlighting specific lesions associated with poor clinical outcomes (for example, driver events such as mutations in the splicing factor subunit gene SF3B1) or with increased clonal heterogeneity (for example, the presence of subclonal driver mutations). Here, we review new genomic discoveries in CLL and discuss their possible implications in the era of precision medicine.
    Preview · Article · May 2013 · Genome Medicine
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