AJHE2306 - CME/CMLE - Progression and transformation of chronic lymphocytic leukemia/ small lymphocytic lymphoma and B-cell prolymphocytic leukemia Report from the 2021 SH/EAHP Workshop-Progression and transformation of chronic lymphocytic leukemia/ small lymphocytic lymphoma and B-cell prolymphocytic leukemia Report from the 2021 SH/EAHP Workshop

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

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The document delves into the progression and transformation of Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma (CLL/SLL) and B-Cell Prolymphocytic Leukemia (B-PLL) as discussed in the 2021 Workshop of the Society for Hematopathology/European Association for Haematopathology. It defines histologic criteria for accelerated CLL/SLL, Richter transformation (RT), and emphasizes genetic testing for prognosis and therapy guidance. Challenges in diagnosing accelerated CLL/SLL in extranodal sites, diverse RT manifestations, clonal relationships, and genetic abnormalities impacting prognosis and treatment outcomes are highlighted.<br /><br />Case studies illustrate the natural progression of CLL/SLL and potential Richter transformations, emphasizing the complexity in diagnosis and management. Genetic abnormalities like MYC alterations and TP53 mutations, EBV reactivation in RT, and distinct morphologic patterns in RT-cHL are discussed. The document stresses the crucial role of molecular genetic studies in enhancing diagnostic accuracy and treatment prediction for CLL/SLL and B-PLL patients.<br /><br />Additionally, insights into various transformations of CLL/SLL, including composite RT into DLBCL and classical Hodgkin lymphoma, PBL in CLL/SLL, T-cell transformations, and B-ALL transformations, are provided. The genetic and molecular dimensions of these transformations, the diagnostic challenges posed by B-PLL, and the importance of advanced molecular genetic testing for precise identification are examined.<br /><br />In conclusion, the document underscores the significance of molecular testing, genetic profiling, and detailed histopathological analysis in comprehending the diverse transformations and progressions in CLL/SLL and B-PLL. These insights aim to enhance diagnostic accuracy, prognostication, and treatment responses for patients with these conditions.

Keywords

Chronic Lymphocytic Leukemia

Small Lymphocytic Lymphoma

B-Cell Prolymphocytic Leukemia

Richter Transformation

Genetic Testing

Prognosis

Therapy Guidance

MYC Alterations

TP53 Mutations

Molecular Genetic Studies