Case report: Targeting the PD-1 receptor and genetic mutations validated in primary histiocytic sarcoma with hemophagocytic lymphohistiocytosis
Histiocytic sarcoma (HS) is a rare hematological malignancy with limited treatment options, and it is also prone to complications such as hemophagocytic lymphohistiocytosis (HLH) in the later stages of the disease, leading to difficulties in treatment and poor prognosis. It highlights the importance...
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| Format: | Article |
| Language: | English |
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Frontiers Media S.A.
2023-03-01
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| Series: | Frontiers in Immunology |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fimmu.2023.1127599/full |
| _version_ | 1811158448219357184 |
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| author | Yan Zhao Yating Deng Yi Jiang Wenli Zheng Yanlin Tan Zhiwu Yang Zhihua Wang Feng Xu Zhao Cheng Lingli Yuan Hongling Peng Hongling Peng |
| author_facet | Yan Zhao Yating Deng Yi Jiang Wenli Zheng Yanlin Tan Zhiwu Yang Zhihua Wang Feng Xu Zhao Cheng Lingli Yuan Hongling Peng Hongling Peng |
| author_sort | Yan Zhao |
| collection | DOAJ |
| description | Histiocytic sarcoma (HS) is a rare hematological malignancy with limited treatment options, and it is also prone to complications such as hemophagocytic lymphohistiocytosis (HLH) in the later stages of the disease, leading to difficulties in treatment and poor prognosis. It highlights the importance of developing novel therapeutic agents. Herein, we present a case of a 45-year-old male patient who was diagnosed with PD-L1-positive HS with HLH. The patient was admitted to our hospital with recurrent high fever, multiple skin rashes with pruritus throughout the body and enlarged lymph nodes. Subsequently, pathological biopsy of the lymph nodes revealed high expression of CD163, CD68, S100, Lys and CD34 in the tumor cells and no expression of CD1a and CD207, confirming this rare clinical diagnosis. Concerning the low remission rate by conventional treatment in this disease, the patient was administered with sintilimab (an anti-programmed cell death 1 [anti-PD-1] monoclonal antibody) at 200 mg/d combined with a first-line chemotherapy regimen for one cycle. Further exploration of pathological biopsy by using next-generation gene sequencing led to the use of targeted therapy of chidamide. After one cycle of combination therapy (chidamide+sintilimab, abbreviated as CS), the patient achieved a favorable response. The patient showed remarkable improvement in the general symptoms and laboratory examination results (e.g., elevated indicators of inflammation); even the clinical benefits was not persistent, he survived one more month after his cessation of treatment by himself due to economic difficulty. Our case suggests that PD-1 inhibitor coupled with targeted therapy might constitute a potential therapeutic option for primary HS with HLH. |
| first_indexed | 2024-04-10T05:23:45Z |
| format | Article |
| id | doaj.art-78f44315e46a436cb82ce045c16fc24e |
| institution | Directory Open Access Journal |
| issn | 1664-3224 |
| language | English |
| last_indexed | 2024-04-10T05:23:45Z |
| publishDate | 2023-03-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Immunology |
| spelling | doaj.art-78f44315e46a436cb82ce045c16fc24e2023-03-08T04:34:01ZengFrontiers Media S.A.Frontiers in Immunology1664-32242023-03-011410.3389/fimmu.2023.11275991127599Case report: Targeting the PD-1 receptor and genetic mutations validated in primary histiocytic sarcoma with hemophagocytic lymphohistiocytosisYan Zhao0Yating Deng1Yi Jiang2Wenli Zheng3Yanlin Tan4Zhiwu Yang5Zhihua Wang6Feng Xu7Zhao Cheng8Lingli Yuan9Hongling Peng10Hongling Peng11Department of Hematology, The Second Xiangya Hospital, Central South University, Changsha, Hunan, ChinaDepartment of Hematology, The Second Xiangya Hospital, Central South University, Changsha, Hunan, ChinaDepartment of Pathology, The Second Xiangya Hospital, Central South University, Changsha, Hunan, ChinaDepartment of Hematology, The Second Xiangya Hospital, Central South University, Changsha, Hunan, ChinaDepartment of Imaging, The Second Xiangya Hospital, Central South University, Changsha, Hunan, ChinaDepartment of Hematology, Yiyang Central Hospital, Yiyang, Hunan, ChinaDepartment of Hematology, The Second Xiangya Hospital, Central South University, Changsha, Hunan, ChinaDepartment of Thyroid and Breast Surgery, The Second Xiangya Hospital, Central South University, Changsha, Hunan, ChinaDepartment of Hematology, The Second Xiangya Hospital, Central South University, Changsha, Hunan, ChinaDepartment of Hematology, The Second Xiangya Hospital, Central South University, Changsha, Hunan, ChinaDepartment of Hematology, The Second Xiangya Hospital, Central South University, Changsha, Hunan, ChinaHunan Engineering Research Center of Cell Immunotherapy for Hematopoietic Malignancies, Changsha, Hunan, ChinaHistiocytic sarcoma (HS) is a rare hematological malignancy with limited treatment options, and it is also prone to complications such as hemophagocytic lymphohistiocytosis (HLH) in the later stages of the disease, leading to difficulties in treatment and poor prognosis. It highlights the importance of developing novel therapeutic agents. Herein, we present a case of a 45-year-old male patient who was diagnosed with PD-L1-positive HS with HLH. The patient was admitted to our hospital with recurrent high fever, multiple skin rashes with pruritus throughout the body and enlarged lymph nodes. Subsequently, pathological biopsy of the lymph nodes revealed high expression of CD163, CD68, S100, Lys and CD34 in the tumor cells and no expression of CD1a and CD207, confirming this rare clinical diagnosis. Concerning the low remission rate by conventional treatment in this disease, the patient was administered with sintilimab (an anti-programmed cell death 1 [anti-PD-1] monoclonal antibody) at 200 mg/d combined with a first-line chemotherapy regimen for one cycle. Further exploration of pathological biopsy by using next-generation gene sequencing led to the use of targeted therapy of chidamide. After one cycle of combination therapy (chidamide+sintilimab, abbreviated as CS), the patient achieved a favorable response. The patient showed remarkable improvement in the general symptoms and laboratory examination results (e.g., elevated indicators of inflammation); even the clinical benefits was not persistent, he survived one more month after his cessation of treatment by himself due to economic difficulty. Our case suggests that PD-1 inhibitor coupled with targeted therapy might constitute a potential therapeutic option for primary HS with HLH.https://www.frontiersin.org/articles/10.3389/fimmu.2023.1127599/fullhistiocytic sarcomahemophagocytic lymphohistiocytosissequencingprogrammed death-ligand 1 (PD-L1)targeted therapy |
| spellingShingle | Yan Zhao Yating Deng Yi Jiang Wenli Zheng Yanlin Tan Zhiwu Yang Zhihua Wang Feng Xu Zhao Cheng Lingli Yuan Hongling Peng Hongling Peng Case report: Targeting the PD-1 receptor and genetic mutations validated in primary histiocytic sarcoma with hemophagocytic lymphohistiocytosis Frontiers in Immunology histiocytic sarcoma hemophagocytic lymphohistiocytosis sequencing programmed death-ligand 1 (PD-L1) targeted therapy |
| title | Case report: Targeting the PD-1 receptor and genetic mutations validated in primary histiocytic sarcoma with hemophagocytic lymphohistiocytosis |
| title_full | Case report: Targeting the PD-1 receptor and genetic mutations validated in primary histiocytic sarcoma with hemophagocytic lymphohistiocytosis |
| title_fullStr | Case report: Targeting the PD-1 receptor and genetic mutations validated in primary histiocytic sarcoma with hemophagocytic lymphohistiocytosis |
| title_full_unstemmed | Case report: Targeting the PD-1 receptor and genetic mutations validated in primary histiocytic sarcoma with hemophagocytic lymphohistiocytosis |
| title_short | Case report: Targeting the PD-1 receptor and genetic mutations validated in primary histiocytic sarcoma with hemophagocytic lymphohistiocytosis |
| title_sort | case report targeting the pd 1 receptor and genetic mutations validated in primary histiocytic sarcoma with hemophagocytic lymphohistiocytosis |
| topic | histiocytic sarcoma hemophagocytic lymphohistiocytosis sequencing programmed death-ligand 1 (PD-L1) targeted therapy |
| url | https://www.frontiersin.org/articles/10.3389/fimmu.2023.1127599/full |
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