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

Hematological Oncology
Idanna InnocentiLuca Laurenti

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.

References

May 20, 2006·Journal of Clinical Oncology : Official Journal of the American Society of Clinical Oncology·Apostolia-Maria TsimberidouMichael J Keating
May 22, 2008·British Journal of Haematology·Davide RossiGianluca Gaidano
Jun 11, 2009·Clinical Cancer Research : an Official Journal of the American Association for Cancer Research·Davide RossiGianluca Gaidano
Jan 24, 2013·Leukemia & Lymphoma·Davide Rossi
Jan 15, 2014·Blood·Sameer A ParikhTait D Shanafelt
Mar 25, 2014·Nature Reviews. Cancer·Rudi W HendriksLaurens P Kil
Sep 1, 2015·Annals of Oncology : Official Journal of the European Society for Medical Oncology·B EichhorstUNKNOWN ESMO Guidelines Committee
Apr 5, 2016·Seminars in Oncology·Davide Rossi, Gianluca Gaidano
Oct 25, 2017·Annals of Hematology·Maliha KhanPhilip A Thompson

❮ Previous
Next ❯

Citations

Jul 7, 2018·Leukemia & Lymphoma·Alankrita TanejaClare Sun
Jan 25, 2020·Annual Review of Pathology·Ferran NadeuElías Campo
Jul 7, 2019·Nature Reviews. Clinical Oncology·Francesc Bosch, Riccardo Dalla-Favera

❮ Previous
Next ❯

Related Concepts

Related Feeds

B-Cell Lymphoma

B-cell lymphomas include lymphomas that affect B cells. This subtype of cancer accounts for over 80% of non-Hodgkin lymphomas in the US. Here is the latest research.

B-Cell Leukemia (Keystone)

B-cell leukemia includes various types of lymphoid leukemia that affect B cells. Here is the latest research on B-cell leukemia.