Sentinel case of Richter transformation from chronic lymphocytic leukaemia/small lymphocytic lymphoma to CD3+ diffuse large B-cell lymphoma. Issue 7 (29th November 2016)

• Record Type: Journal Article
• Authors: Ismail, Ali; Mallick, Jawed A; Qin, Dahui; Hussaini, Mohammad O
• Is Part Of: Journal of clinical pathology. Volume 70:Issue 7(2017)
• Page Start: 575
• Page End: 578
• Publication Date: 2016-11-29
• Subjects: LYMPHOMA -- HEMATOPATHOLOGY -- MOLECULAR PATHOLOGY; Pathology -- Periodicals; Pathology, Molecular -- Periodicals; 616.0705
• DOI: 10.1136/jclinpath-2016-204024
• Languages: English
• ISSNs: 0021-9746

Abstract

Abstract : Aim: To report the first case of a Richter syndrome where small lymphocytic lymphoma (SLL) progressed to a CD3+ diffuse large B-cell lymphoma (DLBCL). Methods: Macrodissection of small and large cell lymphomatous components was performed. This was followed by flow cytometric analysis along with molecular B-cell immunoglobulin (heavy and light chains) and T-cell receptor (γ and β chains) gene rearrangement studies to investigate a clonal relationship between the components. Results: The immunophenotypic profile was similar between small and large cell components of the lymphoma by flow cytometry. Furthermore, shared clonal peaks were observed between both components based on molecular B-cell and T-cell receptor gene rearrangement studies, confirming a clonal relationship. Conclusions: Chronic lymphocytic leukaemia/SLL may rarely undergo Richter transformation to a DLBCL demonstrating lineage infidelity. This is a potentially important diagnostic pitfall and such cases should not be confused with a de novo T-cell lymphoma.