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Sentinel case of Richter transformation from chronic lymphocytic leukaemia/small lymphocytic lymphoma to CD3+ diffuse large B-cell lymphoma
  1. Ali Ismail1,
  2. Jawed A Mallick2,
  3. Dahui Qin2,
  4. Mohammad O Hussaini2
  1. 1University of Pikeville Kentucky College of Osteopathic Medicine, Pikeville, Kentucky, USA
  2. 2Moffitt Cancer Center, Tampa, Florida, USA
  1. Correspondence to Dr Mohammad O Hussaini, Moffitt Cancer Center, 12902 Magnolia Drive, MCC 2071L, Tampa, FL 33612, USA; maismail09{at}gmail.com

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.

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Footnotes

  • AI and JAM contributed equally.

  • Handling editor Mary Frances McMullin

  • Acknowledgement We acknowledge Esoteric Molecular Diagnostics Lab at M2Gen, Tampa, Florida, USA, for their support in providing molecular B-cell and T-cell receptor gene rearrangement testing.

  • Competing interests None.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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