Ir2 leading to complete remission in r/r richter syndrome – a case report

Abdullayev, Emin; Buhl, Christoph; Niederland, Judith; Baurmann, Herrad; Glass, Bertram

doi:10.1016/j.htct.2020.09.084

Hematology, Transfusion and Cell Therapy

ISSN: 2531-1379

Hematology, Transfusion and Cell Therapy is a quarterly scientific publication of the Associação Brasileira de Hematologia, Hemoterapia e Terapia Celular (ABHH), Associazione Italo-Brasiliana di Ematologia (AIBE), Eurasian Hematology Oncology Group (EHOG), and Sociedade Brasileira de Oncologia Pediátrica (SOBOPE).

Hematology, Transfusion and Cell Therapy publishes original articles, review articles and case reports covering various areas in the field of hematology and hemotherapy. The journal was previously published, until 2016, as Revista Brasileira de Hematologia e Hemoterapia.

ISSN print: 2531-1379
ISSN online: 2531-1387

Published by Elsevier Editora Ltda, Rio de Janeiro, Brazil.

Indexed in:

Web of Science, LILACS, SciELO Brazil, ESCI (Web of Science), Scopus, and PubMed/PMC

Introduction: Relapsed and refractory diffuse large B Non-Hodgkin lymphoma (r/r DLBCL) is a severe condition with fatal outcome for the majority of the patients. (1) Richter Syndrome is defined as a transformation of chronic lymphatic leukemia in a highly aggressive B-Non-Hodgkin lymphoma, mainly DLBCL. 20% of Richters Syndromes are de novo DLBCL, implying comparable prognosis to other aggressive Non-Hodgkin Lymphoma, whereas 80% are clonally related to the CLL cells and imply a poor prognosis of one-year median overall survival. (2) Despite huge efforts that have been achieved recently by implementing CAR-T Cells for r/r DLBCL and transformed Follicular Lymphoma, treatment of r/r Richter syndrome remains desperate with poor outcome. Allogenic stem cell transplantation is recommended for eligible patients. The combination of Anti CD 20 Antibody Rituximab with IMiD Lenalidomide and Bruton-kinase inhibitor Ibrutinib iR2 has shown safety and efficacy in a breaking phase II study. (2)

We present the rare case of a patient with refractory DLBCL after CLL (Richter Transformation) who achieved complete remission with iR2 and was successfully transplanted.

Case report: Our by now 74-year old patient was first diagnosed with CLL in 08/2014. He showed ubiquitous lymph nodes and evidence of p53 mutation, Binet stage B & RAI I.

He was treated with Ofatumumab+Bendamustine in the first line, Rituximab+Idelalisib in first relapse and Ibrutinib in second relapse before evolving to highly aggressive B-NHL in 10/2019. Richters Syndrome was first treated with Standard Immunochemotherapy (R-CHOP), before switching to Rituximab+Ifosphamid+Etoposid+Carboplatin (R-ICE) for refractory disease. There was further progress (clearly progressive lymph nodes cervical) after first cycle R-ICE chemotherapy, we decided to treat with a combination of immunotherapy with the Anti CD 79a-Antibody Polatuzumab in combination with Rituximab. Unfortunately, we saw again progressive disease after three cycles, that lead to the decision of experimental application of Ibrutinib in combination with Rituximab and Lenalidomid.

We saw an immediate effect as Lactat-dehydrogenase normalized very soon and lymph nodes disappeared completely.

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