Severe cytokine release syndrome resulting in purpura fulminans despite successful response to nivolumab therapy in a patient with pleomorphic carcinoma of the lung: a case report
Abstract Background Immune checkpoint inhibitors (ICIs) have provided more options in the treatment of lung cancer. However, ICIs can cause several unfavorable reactions generally referred to as immune-related adverse effects. Case presentation In this report, we present the case of a 52-year-old wo...
| Main Authors: | , , , , , , , |
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| Format: | Article |
| Language: | English |
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BMJ Publishing Group
2019-04-01
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| Series: | Journal for ImmunoTherapy of Cancer |
| Online Access: | http://link.springer.com/article/10.1186/s40425-019-0582-4 |
| _version_ | 1828840133155094528 |
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| author | Osamu Honjo Terufumi Kubo Fumiko Sugaya Takahiro Nishizaka Koji Kato Yoshihiko Hirohashi Hiroki Takahashi Toshihiko Torigoe |
| author_facet | Osamu Honjo Terufumi Kubo Fumiko Sugaya Takahiro Nishizaka Koji Kato Yoshihiko Hirohashi Hiroki Takahashi Toshihiko Torigoe |
| author_sort | Osamu Honjo |
| collection | DOAJ |
| description | Abstract Background Immune checkpoint inhibitors (ICIs) have provided more options in the treatment of lung cancer. However, ICIs can cause several unfavorable reactions generally referred to as immune-related adverse effects. Case presentation In this report, we present the case of a 52-year-old woman with successful regression of pleomorphic carcinoma of the lung following nivolumab therapy. She developed purpura fulminans (PF) ultimately resulting in amputation of both lower extremities. Blood tests revealed thrombocytopenia with increased serum soluble IL-2 receptor, ferritin, and triglyceride levels suggesting hemophagocytic lymphohistiocytosis (HLH). In addition, serum A disintegrin-like and metalloproteinase with thrombospondin type 1 motifs 13 activity was decreased, suggesting thrombotic thrombocytopenic purpura (TTP). Further detailed analysis revealed severe hypercytokinemia including increased levels of IL-1β, IL-6, IL-10, TNFα, IFNγ, and G-CSF. Conclusion The severe systemic inflammatory reaction and impaired peripheral circulation in this patient was attributed to excessive immunological effect induced by nivolumab resulting in cytokine release syndrome (CRS). This is the first report of a patient with multiple pathological conditions including HLH, TTP-like condition, and PF presumably arising from ICI-induced CRS. Further accumulating thoroughly investigated cases would lead to better understanding of the disease and development of reliable cancer immunotherapy. |
| first_indexed | 2024-12-12T19:36:53Z |
| format | Article |
| id | doaj.art-c9f505ea2ead4ca18ba92f435e68485d |
| institution | Directory Open Access Journal |
| issn | 2051-1426 |
| language | English |
| last_indexed | 2024-12-12T19:36:53Z |
| publishDate | 2019-04-01 |
| publisher | BMJ Publishing Group |
| record_format | Article |
| series | Journal for ImmunoTherapy of Cancer |
| spelling | doaj.art-c9f505ea2ead4ca18ba92f435e68485d2022-12-22T00:14:18ZengBMJ Publishing GroupJournal for ImmunoTherapy of Cancer2051-14262019-04-01711610.1186/s40425-019-0582-4Severe cytokine release syndrome resulting in purpura fulminans despite successful response to nivolumab therapy in a patient with pleomorphic carcinoma of the lung: a case reportOsamu Honjo0Terufumi Kubo1Fumiko Sugaya2Takahiro Nishizaka3Koji Kato4Yoshihiko Hirohashi5Hiroki Takahashi6Toshihiko Torigoe7Department of Respiratory Medicine, Sapporo Minami-Sanjo HospitalDepartment of Pathology, Sapporo Medical University, School of MedicineDepartment of Respiratory Medicine, Teine Keijinkai HospitalSakaemachi Dermatology ClinicDepartment of Pathology, Sapporo Medical University, School of MedicineDepartment of Pathology, Sapporo Medical University, School of MedicineDepartment of Respiratory Medicine and Allergology, Sapporo Medical University, School of MedicineDepartment of Pathology, Sapporo Medical University, School of MedicineAbstract Background Immune checkpoint inhibitors (ICIs) have provided more options in the treatment of lung cancer. However, ICIs can cause several unfavorable reactions generally referred to as immune-related adverse effects. Case presentation In this report, we present the case of a 52-year-old woman with successful regression of pleomorphic carcinoma of the lung following nivolumab therapy. She developed purpura fulminans (PF) ultimately resulting in amputation of both lower extremities. Blood tests revealed thrombocytopenia with increased serum soluble IL-2 receptor, ferritin, and triglyceride levels suggesting hemophagocytic lymphohistiocytosis (HLH). In addition, serum A disintegrin-like and metalloproteinase with thrombospondin type 1 motifs 13 activity was decreased, suggesting thrombotic thrombocytopenic purpura (TTP). Further detailed analysis revealed severe hypercytokinemia including increased levels of IL-1β, IL-6, IL-10, TNFα, IFNγ, and G-CSF. Conclusion The severe systemic inflammatory reaction and impaired peripheral circulation in this patient was attributed to excessive immunological effect induced by nivolumab resulting in cytokine release syndrome (CRS). This is the first report of a patient with multiple pathological conditions including HLH, TTP-like condition, and PF presumably arising from ICI-induced CRS. Further accumulating thoroughly investigated cases would lead to better understanding of the disease and development of reliable cancer immunotherapy.http://link.springer.com/article/10.1186/s40425-019-0582-4 |
| spellingShingle | Osamu Honjo Terufumi Kubo Fumiko Sugaya Takahiro Nishizaka Koji Kato Yoshihiko Hirohashi Hiroki Takahashi Toshihiko Torigoe Severe cytokine release syndrome resulting in purpura fulminans despite successful response to nivolumab therapy in a patient with pleomorphic carcinoma of the lung: a case report Journal for ImmunoTherapy of Cancer |
| title | Severe cytokine release syndrome resulting in purpura fulminans despite successful response to nivolumab therapy in a patient with pleomorphic carcinoma of the lung: a case report |
| title_full | Severe cytokine release syndrome resulting in purpura fulminans despite successful response to nivolumab therapy in a patient with pleomorphic carcinoma of the lung: a case report |
| title_fullStr | Severe cytokine release syndrome resulting in purpura fulminans despite successful response to nivolumab therapy in a patient with pleomorphic carcinoma of the lung: a case report |
| title_full_unstemmed | Severe cytokine release syndrome resulting in purpura fulminans despite successful response to nivolumab therapy in a patient with pleomorphic carcinoma of the lung: a case report |
| title_short | Severe cytokine release syndrome resulting in purpura fulminans despite successful response to nivolumab therapy in a patient with pleomorphic carcinoma of the lung: a case report |
| title_sort | severe cytokine release syndrome resulting in purpura fulminans despite successful response to nivolumab therapy in a patient with pleomorphic carcinoma of the lung a case report |
| url | http://link.springer.com/article/10.1186/s40425-019-0582-4 |
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