Clinical, pathological, and biological characterization of Richter syndrome developing after ibrutinib treatment for relapsed chronic lymphocytic leukemia
| Autor: | Davide Rossi, Francesco Autore, Paolo Falcucci, Simona Sica, Vincenzo Gomes, Giulio Trapè, Luigi Maria Larocca, Gianluca Gaidano, Idanna Innocenti, Michaela Cerri, Luca Laurenti |
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| Jazyk: | angličtina |
| Rok vydání: | 2018 |
| Předmět: |
0301 basic medicine
Oncology Cancer Research medicine.medical_specialty Chronic lymphocytic leukemia 03 medical and health sciences chemistry.chemical_compound 0302 clinical medicine immune system diseases ibrutinib hemic and lymphatic diseases Internal medicine medicine Bruton's tyrosine kinase neoplasms Pathological PLCG2 Richter biology business.industry Hematology General Medicine medicine.disease Lymphoma Settore MED/15 - MALATTIE DEL SANGUE 030104 developmental biology medicine.anatomical_structure chemistry CLL 030220 oncology & carcinogenesis Ibrutinib biology.protein Bone marrow Complication business |
| Popis: | Richter syndrome, a transformation of chronic lymphocytic leukemia (CLL) into a diffuse large B-cell lymphoma, is a rare complication of patients treated with chemo-immunotherapy. Richter syndrome might be both clonally related or unrelated to the underlying CLL and often showed mutations of the TP53 and NOTCH1 genes. Recently, ibrutinib was approved for patients with relapsed/refractory CLL or for untreated CLL patients with del 17p or TP53 mutation. The clinical picture, pathology, and genetics of Richter transformation after IBR treatment are largely unknown. Here, we report 2 cases of Richter transformation after Ibrutinib treatment. As just reported by previous report, Richter syndrome developing after ibrutinib therapy lacked resistance mutations of the BTK and PLCG2 genes, which are clonally related to the pre-existent CLL phase representing transformation from CLL. Richter syndrome after ibrutinib seems to have some peculiar clinical findings as the bone marrow predilection, severe hypercalcemia, and a more aggressive outcome. |
| Databáze: | OpenAIRE |
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