Bilateral orbital plasmacytomas as first sign of extramedullary progression post CAR-T therapy: case report and literature review

BackgroundPlasma cell leukemia (PCL) is an aggressive and rare form of plasma cell dyscrasia characterized by peripheral blood expression, poor prognosis, and high relapse rates. Extramedullary plasmacytomas are common in this entity and can affect various organs and soft tissues. Chimeric antigen r...

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Main Authors: Javier Nogués-Castell, Silvia Feu-Basilio, Óscar Felguera García, Carlos Fernández de Larrea, Aina Oliver-Caldés, Olga Balagué Ponz, Jessica Matas Fassi
Format: Article
Language:English
Published: Frontiers Media S.A. 2023-08-01
Series:Frontiers in Oncology
Subjects:
Online Access:https://www.frontiersin.org/articles/10.3389/fonc.2023.1217714/full
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author Javier Nogués-Castell
Javier Nogués-Castell
Silvia Feu-Basilio
Silvia Feu-Basilio
Óscar Felguera García
Óscar Felguera García
Carlos Fernández de Larrea
Carlos Fernández de Larrea
Aina Oliver-Caldés
Aina Oliver-Caldés
Olga Balagué Ponz
Olga Balagué Ponz
Jessica Matas Fassi
Jessica Matas Fassi
author_facet Javier Nogués-Castell
Javier Nogués-Castell
Silvia Feu-Basilio
Silvia Feu-Basilio
Óscar Felguera García
Óscar Felguera García
Carlos Fernández de Larrea
Carlos Fernández de Larrea
Aina Oliver-Caldés
Aina Oliver-Caldés
Olga Balagué Ponz
Olga Balagué Ponz
Jessica Matas Fassi
Jessica Matas Fassi
author_sort Javier Nogués-Castell
collection DOAJ
description BackgroundPlasma cell leukemia (PCL) is an aggressive and rare form of plasma cell dyscrasia characterized by peripheral blood expression, poor prognosis, and high relapse rates. Extramedullary plasmacytomas are common in this entity and can affect various organs and soft tissues. Chimeric antigen receptor–T-cell (CAR-T) therapy is a novel immunotherapy for hematological malignancies with promising results. However, it is not indicated for PCL, and experience in this condition is limited. This case is a rare presentation of bilateral orbital plasmacytomas after CAR-T therapy in a patient with PCL history.Case presentationWe present the case of a 51-year-old female patient with a history of previous primary PCL treated with CAR-T therapy achieving complete response and without evidence of systemic progression. Six months after the treatment, she developed subacute proptosis and ptosis on the left eye.An orbital CT scan was performed and showed an orbital tumor in both eyes. A surgical biopsy with histological examination revealed plasma cells, consistent with a plasmacytoma. PET-CT and MRI confirmed the presence of tumors in both orbits. The patient was treated with dexamethasone and chemotherapy along with palliative radiation therapy to the left orbit which had a good response.ConclusionOrbital involvement in multiple myeloma and PCL is rare, with plasmacytomas being more common in other parts of the body. In this report, we present a case of a patient with PCL history, treated with multiple therapeutic lines including CAR-T therapy, who presented bilateral orbital plasmacytomas as the first sign of extramedullary progression after the treatment. This case should be considered by specialist to be aware that the orbits are a possible location of extramedullary progression.
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spelling doaj.art-0e0d1ec5a3954870b2f7786d167338cc2023-08-11T01:21:22ZengFrontiers Media S.A.Frontiers in Oncology2234-943X2023-08-011310.3389/fonc.2023.12177141217714Bilateral orbital plasmacytomas as first sign of extramedullary progression post CAR-T therapy: case report and literature reviewJavier Nogués-Castell0Javier Nogués-Castell1Silvia Feu-Basilio2Silvia Feu-Basilio3Óscar Felguera García4Óscar Felguera García5Carlos Fernández de Larrea6Carlos Fernández de Larrea7Aina Oliver-Caldés8Aina Oliver-Caldés9Olga Balagué Ponz10Olga Balagué Ponz11Jessica Matas Fassi12Jessica Matas Fassi13Institut Clínic d’Oftalmologia, Hospital Clínic de Barcelona, Universitat de Barcelona, Barcelona, SpainInstitut D’ Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Fundació Clínic per a la Recerca Biomèdica (FCRB), Universitat de Barcelona, Barcelona, SpainInstitut Clínic d’Oftalmologia, Hospital Clínic de Barcelona, Universitat de Barcelona, Barcelona, SpainInstitut D’ Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Fundació Clínic per a la Recerca Biomèdica (FCRB), Universitat de Barcelona, Barcelona, SpainInstitut Clínic d’Oftalmologia, Hospital Clínic de Barcelona, Universitat de Barcelona, Barcelona, SpainInstitut D’ Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Fundació Clínic per a la Recerca Biomèdica (FCRB), Universitat de Barcelona, Barcelona, SpainInstitut D’ Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Fundació Clínic per a la Recerca Biomèdica (FCRB), Universitat de Barcelona, Barcelona, SpainAmyloidosis and Myeloma Unit, Department of Hematology, Hospital Clínic de Barcelona, Universitat de Barcelona, Barcelona, SpainInstitut D’ Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Fundació Clínic per a la Recerca Biomèdica (FCRB), Universitat de Barcelona, Barcelona, SpainAmyloidosis and Myeloma Unit, Department of Hematology, Hospital Clínic de Barcelona, Universitat de Barcelona, Barcelona, SpainInstitut D’ Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Fundació Clínic per a la Recerca Biomèdica (FCRB), Universitat de Barcelona, Barcelona, SpainCentre de Diagnòstic Biomèdic, Hospital Clínic de Barcelona, Universitat de Barcelona, Barcelona, SpainInstitut Clínic d’Oftalmologia, Hospital Clínic de Barcelona, Universitat de Barcelona, Barcelona, SpainInstitut D’ Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Fundació Clínic per a la Recerca Biomèdica (FCRB), Universitat de Barcelona, Barcelona, SpainBackgroundPlasma cell leukemia (PCL) is an aggressive and rare form of plasma cell dyscrasia characterized by peripheral blood expression, poor prognosis, and high relapse rates. Extramedullary plasmacytomas are common in this entity and can affect various organs and soft tissues. Chimeric antigen receptor–T-cell (CAR-T) therapy is a novel immunotherapy for hematological malignancies with promising results. However, it is not indicated for PCL, and experience in this condition is limited. This case is a rare presentation of bilateral orbital plasmacytomas after CAR-T therapy in a patient with PCL history.Case presentationWe present the case of a 51-year-old female patient with a history of previous primary PCL treated with CAR-T therapy achieving complete response and without evidence of systemic progression. Six months after the treatment, she developed subacute proptosis and ptosis on the left eye.An orbital CT scan was performed and showed an orbital tumor in both eyes. A surgical biopsy with histological examination revealed plasma cells, consistent with a plasmacytoma. PET-CT and MRI confirmed the presence of tumors in both orbits. The patient was treated with dexamethasone and chemotherapy along with palliative radiation therapy to the left orbit which had a good response.ConclusionOrbital involvement in multiple myeloma and PCL is rare, with plasmacytomas being more common in other parts of the body. In this report, we present a case of a patient with PCL history, treated with multiple therapeutic lines including CAR-T therapy, who presented bilateral orbital plasmacytomas as the first sign of extramedullary progression after the treatment. This case should be considered by specialist to be aware that the orbits are a possible location of extramedullary progression.https://www.frontiersin.org/articles/10.3389/fonc.2023.1217714/fullplasma cells leukemiachimeric antigen receptor therapyCAR-Torbital plasmacytomaorbital multiple myeloma
spellingShingle Javier Nogués-Castell
Javier Nogués-Castell
Silvia Feu-Basilio
Silvia Feu-Basilio
Óscar Felguera García
Óscar Felguera García
Carlos Fernández de Larrea
Carlos Fernández de Larrea
Aina Oliver-Caldés
Aina Oliver-Caldés
Olga Balagué Ponz
Olga Balagué Ponz
Jessica Matas Fassi
Jessica Matas Fassi
Bilateral orbital plasmacytomas as first sign of extramedullary progression post CAR-T therapy: case report and literature review
Frontiers in Oncology
plasma cells leukemia
chimeric antigen receptor therapy
CAR-T
orbital plasmacytoma
orbital multiple myeloma
title Bilateral orbital plasmacytomas as first sign of extramedullary progression post CAR-T therapy: case report and literature review
title_full Bilateral orbital plasmacytomas as first sign of extramedullary progression post CAR-T therapy: case report and literature review
title_fullStr Bilateral orbital plasmacytomas as first sign of extramedullary progression post CAR-T therapy: case report and literature review
title_full_unstemmed Bilateral orbital plasmacytomas as first sign of extramedullary progression post CAR-T therapy: case report and literature review
title_short Bilateral orbital plasmacytomas as first sign of extramedullary progression post CAR-T therapy: case report and literature review
title_sort bilateral orbital plasmacytomas as first sign of extramedullary progression post car t therapy case report and literature review
topic plasma cells leukemia
chimeric antigen receptor therapy
CAR-T
orbital plasmacytoma
orbital multiple myeloma
url https://www.frontiersin.org/articles/10.3389/fonc.2023.1217714/full
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