Leukemia escape in immune desert: intraocular relapse of pediatric pro-B-ALL during systemic control by CD19-CAR T cells

Background Relapsed/refractory B-precursor acute lymphoblastic leukemia (BCP-ALL) remains a major therapeutic challenge in pediatric hematology. Chimeric antigen receptor (CAR) T cells targeting CD19 have shown remarkable initial response rates in BCP-ALL patients, while long-term leukemia control r...

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Main Authors: Armin Wolf, Semjon Willier, Johannes Raedler, Franziska Blaeschke, Dana Stenger, Montserrat Pazos Escudero, Florian Jurgeleit, Thomas G P Grünewald, Vera Binder, Irene Schmid, Michael H Albert, Tobias Feuchtinger
Format: Article
Language:English
Published: BMJ Publishing Group 2020-10-01
Series:Journal for ImmunoTherapy of Cancer
Online Access:https://jitc.bmj.com/content/8/2/e001052.full
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author Armin Wolf
Semjon Willier
Johannes Raedler
Franziska Blaeschke
Dana Stenger
Montserrat Pazos Escudero
Florian Jurgeleit
Thomas G P Grünewald
Vera Binder
Irene Schmid
Michael H Albert
Tobias Feuchtinger
author_facet Armin Wolf
Semjon Willier
Johannes Raedler
Franziska Blaeschke
Dana Stenger
Montserrat Pazos Escudero
Florian Jurgeleit
Thomas G P Grünewald
Vera Binder
Irene Schmid
Michael H Albert
Tobias Feuchtinger
author_sort Armin Wolf
collection DOAJ
description Background Relapsed/refractory B-precursor acute lymphoblastic leukemia (BCP-ALL) remains a major therapeutic challenge in pediatric hematology. Chimeric antigen receptor (CAR) T cells targeting CD19 have shown remarkable initial response rates in BCP-ALL patients, while long-term leukemia control rate is only about 50%. So far, main mechanisms of BCP-ALL relapse after CD19-CAR T-cell therapy have been either insufficient CAR T-cell persistence in vivo or loss of surface CD19.Case Report Here, we report an exceptional presentation of BCP-ALL relapse in the eye during the systemic control through CAR T-cell therapy. We report a case of fatal intraocular relapse in a pediatric patient with pro-B-ALL after initial response to CD19-CAR T-cell therapy. One month after CD19-CAR T-cell therapy, remission was documented by bone marrow aspirate analysis with absence of CD19+ cells and CD19-CAR T cells could be detected in both peripheral blood and bone marrow. At the same time, however, the patient presented with progressive visual disturbance and CD19+ cells were found within the anterior chamber of the eye. Despite local and systemic therapy, ocular relapse led to BCP-ALL dissemination and systemic relapse within weeks. The eye represents a rare site for local manifestation of BCP-ALL, but isolated intraocular relapse is a clinically unreckoned presentation of BCP-ALL in the era of CD19-CAR T cells.Conclusion During systemic control of BCP-ALL through CD19-CAR T cells, relapse can emerge in the eye as an immune-privileged organ. Ocular symptoms after CD19-CAR T-cell therapy should guide the clinician to elucidate the etiology in a timely fashion in order to adjust leukemia treatment strategy. Both, local immune escape as well as insufficient CAR T-cell persistence may have contributed to relapse in the reported patient. Mechanisms of relapse in an immune desert under CAR T-cell therapy require future clinical and experimental attention. In particular, ocular symptoms after CAR T-cell therapy should be considered a potentially early sign of leukemia relapse.
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spelling doaj-art-27c8b25d5ad94f20ab2fed383e907a262024-11-10T02:10:08ZengBMJ Publishing GroupJournal for ImmunoTherapy of Cancer2051-14262020-10-018210.1136/jitc-2020-001052Leukemia escape in immune desert: intraocular relapse of pediatric pro-B-ALL during systemic control by CD19-CAR T cellsArmin Wolf0Semjon Willier1Johannes Raedler2Franziska Blaeschke3Dana Stenger4Montserrat Pazos Escudero5Florian Jurgeleit6Thomas G P Grünewald7Vera Binder8Irene Schmid9Michael H Albert10Tobias Feuchtinger11Department of Ophthalmology, Ulm University Medical Center, Ulm, Germany1 Pediatric Hematology, Oncology, Hemostaseology and Stem Cell Transplantation, Dr. von Hauner Children’s Hospital, University Hospital, LMU Munich, Munich, Bavaria, Germany1 Pediatric Hematology, Oncology, Hemostaseology and Stem Cell Transplantation, Dr. von Hauner Children’s Hospital, University Hospital, LMU Munich, Munich, Bavaria, Germany1 Pediatric Hematology, Oncology, Hemostaseology and Stem Cell Transplantation, Dr. von Hauner Children’s Hospital, University Hospital, LMU Munich, Munich, Bavaria, Germany1 Pediatric Hematology, Oncology, Hemostaseology and Stem Cell Transplantation, Dr. von Hauner Children’s Hospital, University Hospital, LMU Munich, Munich, Bavaria, Germany3 Department of Radiotherapy, University Hospital Munich, Ludwig Maximilian University Munich, Munich, Germany1 Pediatric Hematology, Oncology, Hemostaseology and Stem Cell Transplantation, Dr. von Hauner Children’s Hospital, University Hospital, LMU Munich, Munich, Bavaria, Germany2 German Cancer Research Center (DKFZ), Heidelberg, Germany1 Pediatric Hematology, Oncology, Hemostaseology and Stem Cell Transplantation, Dr. von Hauner Children’s Hospital, University Hospital, LMU Munich, Munich, Bavaria, Germany1 Pediatric Hematology, Oncology, Hemostaseology and Stem Cell Transplantation, Dr. von Hauner Children’s Hospital, University Hospital, LMU Munich, Munich, Bavaria, Germany1 Pediatric Hematology, Oncology, Hemostaseology and Stem Cell Transplantation, Dr. von Hauner Children’s Hospital, University Hospital, LMU Munich, Munich, Bavaria, Germany1 Pediatric Hematology, Oncology, Hemostaseology and Stem Cell Transplantation, Dr. von Hauner Children’s Hospital, University Hospital, LMU Munich, Munich, Bavaria, GermanyBackground Relapsed/refractory B-precursor acute lymphoblastic leukemia (BCP-ALL) remains a major therapeutic challenge in pediatric hematology. Chimeric antigen receptor (CAR) T cells targeting CD19 have shown remarkable initial response rates in BCP-ALL patients, while long-term leukemia control rate is only about 50%. So far, main mechanisms of BCP-ALL relapse after CD19-CAR T-cell therapy have been either insufficient CAR T-cell persistence in vivo or loss of surface CD19.Case Report Here, we report an exceptional presentation of BCP-ALL relapse in the eye during the systemic control through CAR T-cell therapy. We report a case of fatal intraocular relapse in a pediatric patient with pro-B-ALL after initial response to CD19-CAR T-cell therapy. One month after CD19-CAR T-cell therapy, remission was documented by bone marrow aspirate analysis with absence of CD19+ cells and CD19-CAR T cells could be detected in both peripheral blood and bone marrow. At the same time, however, the patient presented with progressive visual disturbance and CD19+ cells were found within the anterior chamber of the eye. Despite local and systemic therapy, ocular relapse led to BCP-ALL dissemination and systemic relapse within weeks. The eye represents a rare site for local manifestation of BCP-ALL, but isolated intraocular relapse is a clinically unreckoned presentation of BCP-ALL in the era of CD19-CAR T cells.Conclusion During systemic control of BCP-ALL through CD19-CAR T cells, relapse can emerge in the eye as an immune-privileged organ. Ocular symptoms after CD19-CAR T-cell therapy should guide the clinician to elucidate the etiology in a timely fashion in order to adjust leukemia treatment strategy. Both, local immune escape as well as insufficient CAR T-cell persistence may have contributed to relapse in the reported patient. Mechanisms of relapse in an immune desert under CAR T-cell therapy require future clinical and experimental attention. In particular, ocular symptoms after CAR T-cell therapy should be considered a potentially early sign of leukemia relapse.https://jitc.bmj.com/content/8/2/e001052.full
spellingShingle Armin Wolf
Semjon Willier
Johannes Raedler
Franziska Blaeschke
Dana Stenger
Montserrat Pazos Escudero
Florian Jurgeleit
Thomas G P Grünewald
Vera Binder
Irene Schmid
Michael H Albert
Tobias Feuchtinger
Leukemia escape in immune desert: intraocular relapse of pediatric pro-B-ALL during systemic control by CD19-CAR T cells
Journal for ImmunoTherapy of Cancer
title Leukemia escape in immune desert: intraocular relapse of pediatric pro-B-ALL during systemic control by CD19-CAR T cells
title_full Leukemia escape in immune desert: intraocular relapse of pediatric pro-B-ALL during systemic control by CD19-CAR T cells
title_fullStr Leukemia escape in immune desert: intraocular relapse of pediatric pro-B-ALL during systemic control by CD19-CAR T cells
title_full_unstemmed Leukemia escape in immune desert: intraocular relapse of pediatric pro-B-ALL during systemic control by CD19-CAR T cells
title_short Leukemia escape in immune desert: intraocular relapse of pediatric pro-B-ALL during systemic control by CD19-CAR T cells
title_sort leukemia escape in immune desert intraocular relapse of pediatric pro b all during systemic control by cd19 car t cells
url https://jitc.bmj.com/content/8/2/e001052.full
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