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


Authors: Czader, M.; Amador, C.; Cook, J. R.; Thakkar, D.; Parker, C.; Dave, S. S.; Dogan, A.; Duffield, A. S.; Nejati, R.; Ott, G.; Xiao, W.; Wasik, M.; Goodlad, J. R.
Review Title: Progression and transformation of chronic lymphocytic leukemia/small lymphocytic lymphoma and B-cell prolymphocytic leukemia: Report from the 2021 SH/EAHP workshop
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. © The Author(s) 2023. Published by Oxford University Press on behalf of American Society for Clinical Pathology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.
Keywords: genetics; pathology; cell transformation, neoplastic; b cell lymphoma; lymphoma, b-cell; progression; chronic lymphatic leukemia; leukemia, lymphocytic, chronic, b-cell; transformation; chronic lymphocytic leukemia; prolymphocytic leukemia; clonal evolution; small lymphocytic lymphoma; humans; human; richter transformation; accelerated phase; neoplastic cell transformation; b-cell prolymphocytic leukemia; leukemia, prolymphocytic, b-cell
Journal Title: American Journal of Clinical Pathology
Volume: 159
Issue: 6
ISSN: 0002-9173
Publisher: Oxford University Press  
Date Published: 2023-06-01
Start Page: 554
End Page: 571
Language: English
DOI: 10.1093/ajcp/aqad027
PUBMED: 37052539
PROVIDER: scopus
PMCID: PMC10233402
DOI/URL:
Notes: The MSK Cancer Center Support Grant (P30 CA008748) is acknowledged in the PDF -- Source: Scopus
Altmetric
Citation Impact
BMJ Impact Analytics
MSK Authors
  1. Ahmet Dogan
    454 Dogan
  2. Wenbin Xiao
    108 Xiao