Progression and transformation of chronic lymphocytic leukemia/ small lymphocytic lymphoma and B-cell prolymphocytic leukemia: Report from the 2021 SH/eAHP workshop

Magdalena Czader, Catalina Amador, James R. Cook, Devang Thakkar, Clay Parker, Sandeep S. Dave, Ahmet Dogan, Amy S. Duffield, Reza Nejati, German Ott, Wenbin Xiao, Mariusz Wasik, John R. Goodlad

Research output: Contribution to journalReview articlepeer-review

1 Scopus citations

Abstract

Objectives: Session 3 of the 2021 Workshop of the Society for Hematopathology/ European Association for Haematopathology examined progression and transformation of chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) and B-cell prolymphocytic leukemia (B-PLL). Methods: Thirty-one cases were reviewed by the panel. Additional studies such as immunohistochemistry and molecular genetic testing, including whole-exome sequencing and expression profiling, were performed in select cases. Results: Session 3 included 27 CLL/SLL cases and miscellaneous associated proliferations, 3 cases of B-PLL, and 1 case of small B-cell lymphoma. The criteria for accelerated CLL/SLL are established for lymph nodes, but extranodal disease can be diagnostically challenging. Richter transformation (RT) is a broad term and includes true transformation from original CLL/SLL clone(s) and clonally unrelated neoplasms. The morphologic, immunophenotypic, and genetic spectrum is diverse with classical and highly unusual examples. T-cell proliferations can also be encountered in CLL/SLL. B-cell prolymphocytic leukemia is a rare, diagnostically challenging disease due to its overlaps with other lymphoid neoplasms. Conclusions: The workshop highlighted complexity of progression and transformation in CLL/SLL and B-PLL, as well as diagnostic caveats accompanying heterogeneous presentations of RT and other manifestations of disease progression. Molecular genetic studies are pivotal for diagnosis and determination of clonal relationship, and to predict response to treatment and identify resistance to targeted therapy.

Original languageEnglish
Pages (from-to)554-571
Number of pages18
JournalAmerican Journal of Clinical Pathology
Volume159
Issue number6
DOIs
StatePublished - Jun 1 2023

Keywords

  • Accelerated phase
  • B-cell prolymphocytic leukemia
  • Chronic lymphocytic leukemia
  • Clonal evolution
  • Progression
  • Richter transformation
  • Small lymphocytic lymphoma
  • Transformation

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