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Chronic Leukemias
Published in Harold R. Schumacher, William A. Rock, Sanford A. Stass, Handbook of Hematologic Pathology, 2019
Scott J. Graham, James D. Cotelingam
Richter’s syndrome (or Richter’s transformation), first described in 1928, is defined as the development of a large cell lymphoma during the course of chronic lymphocytic leukemia (CLL). Richter’s transformation occurs in about 8% of CLL patients, and can be suspected when a CLL patient suddenly develops unexplained weight loss, fever, organomegaly, and rapidly increasing lymphadenopathy.
Specific Therapy for Lymphomas
Published in Tariq I Mughal, John M Goldman, Sabena T Mughal, Understanding Leukemias, Lymphomas, and Myelomas, 2017
Tariq I Mughal, John M Goldman, Sabena T Mughal
DLBCL is not only a heterogenous disease, as discussed in chapter 5, but survival after treatment is influenced by differences in immune cells, fibrosis, and angiogenesis. This was recently demonstrated by an elegant gene expression profiling study of pre- and post-therapy (chemotherapy or immuno- chemotherapy) biopsy specimens. This sheds some light of the different survival times sometimes seen in patients with similar risk-stratification by the revised IPI score (see “Glossary”). DLBCL can also arise from other B-cell lymphomas, in particular follicular lymphomas. It can also arise from CLL/small lymphocytic lymphoma, when the eponym Richter’s transformation is used. Patients with transformed DLBCL tend to fare worse with most conventional therapies used for the treatment of DLBCL.
Practice Paper 5: Answers
Published in Anthony B. Starr, Hiruni Jayasena, David Capewell, Saran Shantikumar, Get ahead! Medicine, 2016
Anthony B. Starr, Hiruni Jayasena, David Capewell
Chronic lymphocytic leukaemia (CLL) accounts for 30% of leukaemias in the over-50s. It is characterized by the accumulation of incompetent CD5+ B cells that fail to secrete antibody (instead secreting light chains). Patients present with anaemia, painless lymphadenopathy, splenomegaly and infection. A blood film reveals a mature lymphocytosis (with anaemia and thrombocytopenia). The median survival is 6 years. CLL can occasionally transform into a high-grade aggressive lymphoma (Richter’s transformation).
Orbital marginal cell lymphoma and mantle cell lymphoma subclone in patient with monoclonal gammopathy of unknown significance
Published in Orbit, 2021
Sitara H. Hirji, Michelle M. Maeng, Andrea A. Tooley, Craig Soderquist, Joseph Annunziata, Michael Kazim
Virtually all indolent lymphomas have the potential to transform into more aggressive phenotypes. Richter’s transformation is the most common, wherein chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) transforms to diffuse large B-cell lymphoma (DLBCL).3 Less commonly, low-grade lymphomas may show Hodgkin-like transformation4−.6 In these cases, the clonal relationship of the synchronous or metachronous low-grade and high-grade lymphomas can be established by demonstrating similarly sized clonal products on polymerase chain reaction (PCR) analysis of immunoglobulin heavy chain (IGH) locus. For cases in which both low-grade and high-grade lymphomas are present, treatment is targeted to the more aggressive histology.4–6 Targeting a more aggressive lymphoma may result in the transformation to a lower grade lymphoma.7
Mechanisms of ibrutinib resistance in chronic lymphocytic leukemia and alternative treatment strategies
Published in Expert Review of Hematology, 2020
Tsering Gyalpo Lama, Daniel Kyung, Susan O’Brien
Richter syndrome (RS) is the development of a fast-growing aggressive lymphoma in patients with CLL [133]. About 2% to 10% of CLL patients will develop RS during the course of their disease. Diffuse large B cell lymphoma (90%) is the most common histology seen in patients with RT, with a small minority transforming to Hodgkin lymphoma or other non‐Hodgkin lymphomas [133,134]. It was observed that RT occurs early in ibrutinib treatment (as opposed to late relapses, which are usually progressive CLL) and is not associated with mutations in BTK and PLCG2 [57,64]. Patients with Richter transformation (RT) have poor outcomes [133,135]. A standard treatment regimen such as R-EPOCH or Hyper-CVAD could be added to ibrutinib if no mutations are identified, but clinical trials are needed to evaluate the efficacy of such combination regimens. The role of novel agents in RS treatment has not yet been explored and is restricted to a few case studies [136–139]. In the ACE-CL-001 clinical study, acalabrutinib was used for treatment of Richter transformation and showed an objective response rate (ORR) of 38.1% [139]. The preliminary finding showed three of six RT patients previously treated with ibrutinib had PR, but we need to wait for final, long-term follow-up result. Early results from recent studies of checkpoint inhibitors and CAR-T cell therapy were encouraging [114,128]. Transplant can be considered for patients with RT especially as remissions after current regimens are generally short-lived [133–135,140]. Because of suboptimal response with traditional treatment, RT patients should always be offered clinical trials.
Cumulative experience and long term follow-up of pentostatin-based chemoimmunotherapy trials for patients with chronic lymphocytic leukemia
Published in Expert Review of Hematology, 2018
Neil E. Kay, Betsy R. LaPlant, Adam M. Pettinger, Timothy G. Call, Jose F. Leis, Wei Ding, Sameer A. Parikh, Michael J. Conte, Deborah A. Bowen, Tait D. Shanafelt
When considering only data reported on the case report forms and entered into the clinical trials database, no incidences of AML or MDS and three incidences of Richter’s transformation were reported during treatment or long-term follow up after discontinuation of treatment. To verify the completeness of reporting for AML/MDS and to evaluate for secondary hematologic malignancies and Richter’s transformation, the patient medical records for all 183 patients who were enrolled at Mayo Clinic were further reviewed. Of the 183 patients in this cohort, 111 are still alive with a median follow up of 6.4 years (Interquartile range 25–75%: 4.8–8.5 years). It was confirmed that no incidences of AML or MDS occurred when including the medical record review. However, an additional 10 patients were found to have had Richter’s transformation for a total of 13 patients overall with Richter’s transformation. In addition, 2 patients with Hodgkin’s disease and 1 patient with acute lymphoblastic leukemia (ALL) were identified during medical record review. Given the accessible records, we cannot rule out that the 2 patients with Hodgkin’s disease met criteria for transformation and in the one ALL patient no Lenalidomide was given to this patient prior to or after development of ALL [18]. Other non-hematologic malignancies noted in these patients are listed in Table 5.