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Innocenti I, Rossi D, Trapè G, et al. Clinical, pathological, and biological characterization of Richter syndrome developing after ibrutinib treatment for relapsed chronic lymphocytic leukemia. Hematol Oncol. 2018;doi: 10.1002/hon.2502.
Innocenti, I., Rossi, D., Trapè, G., Autore, F., Larocca, L. M., Gomes, V., Cerri, M., Falcucci, P., Sica, S., Gaidano, G., & Laurenti, L. (2018). Clinical, pathological, and biological characterization of Richter syndrome developing after ibrutinib treatment for relapsed chronic lymphocytic leukemia. Hematological oncology, . https://doi.org/10.1002/hon.2502
Innocenti, Idanna, et al. "Clinical, pathological, and biological characterization of Richter syndrome developing after ibrutinib treatment for relapsed chronic lymphocytic leukemia." Hematological oncology vol. (2018). doi: https://doi.org/10.1002/hon.2502
Innocenti I, Rossi D, Trapè G, Autore F, Larocca LM, Gomes V, Cerri M, Falcucci P, Sica S, Gaidano G, Laurenti L. Clinical, pathological, and biological characterization of Richter syndrome developing after ibrutinib treatment for relapsed chronic lymphocytic leukemia. Hematol Oncol. 2018 Feb 27; doi: 10.1002/hon.2502. Epub 2018 Feb 27. PMID: 29484684.
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Hematol Oncol. 2018 Feb 27; doi: 10.1002/hon.2502. Epub 2018 Feb 27.

Clinical, pathological, and biological characterization of Richter syndrome developing after ibrutinib treatment for relapsed chronic lymphocytic leukemia.

Hematological oncology

Idanna Innocenti, Davide Rossi, Giulio Trapè, Francesco Autore, Luigi Maria Larocca, Vincenzo Gomes, Michaela Cerri, Paolo Falcucci, Simona Sica, Gianluca Gaidano, Luca Laurenti

Affiliations

  1. Institue of Hematology, Università Cattolica del Sacro Cuore, Rome, Italy.
  2. Institute of Oncology Research and Oncology Institute of Southern Switzerland, Bellinzona, Switzerland.
  3. Division of Hematology, Ospedale Belcolle, Viterbo, Italy.
  4. Institute of Pathology, Università Cattolica del Sacro Cuore, Rome, Italy.
  5. Division of Patology, Ospedale Belcolle, Viterbo, Italy.
  6. Division of Hematology, Department of Translational Medicine, Amedeo Avogadro, University of Eastern Piedmont, Novara, Italy.

PMID: 29484684 DOI: 10.1002/hon.2502

Abstract

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.

Copyright © 2018 John Wiley & Sons, Ltd.

Keywords: CLL; Richter; ibrutinib

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