Richter’s transformation in chronic lymphocytic leukemia
ABSTRACT 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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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.
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ABSTRACT: We present a case of a 45-year-old woman with primary Sjögren's syndrome, who in 2005, one year after the onset of the systemic autoimmune disease developed chronic lymphocytic leukaemia, which eventually transformed -denoted as Richter's transformation -into diffuse large B-cell lymphoma in 2006. She had recurrent lymphadenopathy on the neck region from 2002. In September 2005 anaemia and elevated LDH enzyme levels were detected with persistent subfebrility and lymphatic node enlargement on her neck and axillary region. Chest and abdominal CT, biopsy from the lymphoid glands and crista biopsy were performed and B-CLL was diagnosed, therefore chlorambucil treatment was ad-ministered. After two cycles of chlorambucil the pulmonary involvement extended, the patient had high fever and the ab-dominal CT showed the appearance of multiple hypodens lesions in the liver. Based on the histological evaluation of the hypodens lesions of the liver, the diagnosis of diffuse large B-cell lymphoma has been established, therefore 8 cycles of R-CHOP treatment were administered. The patient has been in complete remission since then. Conclusion: The main message of this case report is that lymphadenopathy, the decrease of serum immunoglobulin levels and autoantibody titres, as well as the predominance of monoclonal gammopathy point to the possibility of malignant transformation in patients with Sjögren's syndrome.
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ABSTRACT: This study examined the ability of the semi-synthetic vinca alkaloid, Vinorelbine/Navelbine, to cause apoptotic death in freshly obtained primary leukaemia cells from 53 patients with haematological malignancies, including 22 patients with acute lymphoblastic leukaemia (ALL), 24 patients with chronic lymphocytic leukaemia (CLL), three patients with chronic myeloid leukaemia in blast crisis (CML-BC) and four patients with acute myeloid leukaemia (AML). Vinorelbine caused apoptosis in primary leukaemia cells from 42 (79%) of these leukaemia patients. Objective responses, including complete remission (CR) and CR with incomplete haematological recovery, were achieved in 12 of 17 (71%) patients with aggressive and therapy-refractory leukaemias, including five of nine patients with relapsed ALL, three of three patients with CML-BC and four of five patients with rapidly progressive CLL, who were treated with a vinorelbine-based salvage chemotherapy regimen. Drug sensitivity profiling of multidrug-resistant primary cancer cells using apoptosis assays revealed a significant association between Vinorelbine sensitivity in vitro and the likelihood of an objective clinical response to Vinorelbine-based chemotherapy. Vinorelbine-sensitivity testing of primary leukaemia cells might help tailor Vinorelbine-based salvage regimens to those patients who are most likely to respond.British Journal of Haematology 12/2006; 135(4):500-8. DOI:10.1111/j.1365-2141.2006.06338.x · 4.96 Impact Factor