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: Richter's syndrome (RS) is associated with poor outcome. The prognosis of patients with histologically aggressive chronic lymphocytic leukemia (CLL), HAC, has not been studied. We aimed to correlate 2-Deoxy-2-[(18)F]fluoroglucose/positron emission tomography (FDG/PET) data, histological diagnosis, clinical characteristics and survival in patients with CLL. 332 patients with CLL were histologically classified as: 95 RS, 117 HAC, and 120 histologically indolent CLL (HIC). HAC and RS patients had higher maximum standardized uptake value (SUVmax), more frequent constitutional symptoms, poorer PS, lower hemoglobin and platelets, higher LDH and beta-2-microglobulin. A SUVmax≥10 strongly correlated with mortality (overall survival, OS, 56.7 vs 6.9 months in patients with SUVmax<10 vs ≥10). Survival of patients with RS and HAC was similar among patients with SUVmax<10 or ≥10. SUVmax≥10, PS≥2, bulky disease, and age≥65 were independently associated with shorter OS. In patients undergoing both fine-needle aspiration (FNA) and biopsy, FNA proved diagnostically inadequate in 23%, 29% and 53% of HIC, HAC and RS, respectively. FDG/PET is a useful diagnostic tool in patients with CLL and suspected transformation. Patients with HAC show different characteristics and worse prognosis compared to those with HIC. Patients with different CLL phases, but similar SUVmax have similar outcome. Tissue biopsy should be preferred for diagnosing RS.Blood 03/2014; · 9.78 Impact Factor
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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.Molecular imaging and radionuclide therapy. 06/2014; 23(2):67-9.
- Leukemia & lymphoma 02/2014; · 2.61 Impact Factor