A Patient with Sequential Diseases of Langerhans Cell Sarcoma, Langerhans Cell Histiocytosis, and Acute Lymphoblastic Leukemia
Langerhans cell histiocytosis(LCH)and Langerhans cell sarcoma(LCS)are characterized by clone proliferation of Langerhans-type cells, which may occur concurrently or sequentially with T-cell acute lymphoblastic leukemia (T-ALL) and other Lymphoid neoplasms. A 15-year old female patient diagnosed with...
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Editorial Office of Journal of Rare Diseases
2022-07-01
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Series: | 罕见病研究 |
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Online Access: | https://jrd.chard.org.cn/article/doi/10.12376/j.issn.2097-0501.2022.03.013 |
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author | TIAN Yu WANG Dong WEI Ang YANG Ying ZHANG Liping MA Honghao WANG Chanjuan CUI Lei LI Zhigang ZHANG Rui WANG Tianyou |
author_facet | TIAN Yu WANG Dong WEI Ang YANG Ying ZHANG Liping MA Honghao WANG Chanjuan CUI Lei LI Zhigang ZHANG Rui WANG Tianyou |
author_sort | TIAN Yu |
collection | DOAJ |
description | Langerhans cell histiocytosis(LCH)and Langerhans cell sarcoma(LCS)are characterized by clone proliferation of Langerhans-type cells, which may occur concurrently or sequentially with T-cell acute lymphoblastic leukemia (T-ALL) and other Lymphoid neoplasms. A 15-year old female patient diagnosed with T-ALL developed LCH involving multiple systems during maintenance chemotherapy of T-AL. After treated with chemotherapy with improved result, the patient showed progression of the illness and refractory to the second-line treatment. We found c.G35A (p.G12D)mutation in the KRAS gene and used the targeted drug Trametinib for treatment. The treatment proved effective, leading to partial remission within a week. Three months after Trametinib treatment, the patient developed new lymphadenopathy. Biopsy revealed the existence of LCS. The disease progressed quickly, and the patient died 7 days after diagnosis of LCS. The case of patients with T-ALL then developing LCH and LCS sequentially is extraordinarily rare. The causes of the case is unclear and may be related to cell transdifferentiation, clonal evolution, and chemotherapy. Targeted drugs can contain this disease for a short time. |
first_indexed | 2024-03-08T17:52:22Z |
format | Article |
id | doaj.art-a0ca844e1b874dd9a19022378583cf9d |
institution | Directory Open Access Journal |
issn | 2097-0501 |
language | zho |
last_indexed | 2024-03-08T17:52:22Z |
publishDate | 2022-07-01 |
publisher | Editorial Office of Journal of Rare Diseases |
record_format | Article |
series | 罕见病研究 |
spelling | doaj.art-a0ca844e1b874dd9a19022378583cf9d2024-01-02T06:57:02ZzhoEditorial Office of Journal of Rare Diseases罕见病研究2097-05012022-07-011331131710.12376/j.issn.2097-0501.2022.03.013A Patient with Sequential Diseases of Langerhans Cell Sarcoma, Langerhans Cell Histiocytosis, and Acute Lymphoblastic LeukemiaTIAN YuWANG Dong0WEI Ang1YANG Ying2ZHANG Liping3MA Honghao4WANG Chanjuan5CUI Lei6LI Zhigang7ZHANG Rui8WANG Tianyou9Hematology Center, National Center for Children's Health, Beijing Children's Hospital, Capital Medical University, Beijing 100045, ChinaHematology Center, National Center for Children's Health, Beijing Children's Hospital, Capital Medical University, Beijing 100045, ChinaHematology Center, National Center for Children's Health, Beijing Children's Hospital, Capital Medical University, Beijing 100045, ChinaHematology Center, National Center for Children's Health, Beijing Children's Hospital, Capital Medical University, Beijing 100045, ChinaHematology Center, National Center for Children's Health, Beijing Children's Hospital, Capital Medical University, Beijing 100045, ChinaHematology Center, National Center for Children's Health, Beijing Children's Hospital, Capital Medical University, Beijing 100045, ChinaHematology Center, National Center for Children's Health, Beijing Children's Hospital, Capital Medical University, Beijing 100045, ChinaHematology Center, National Center for Children's Health, Beijing Children's Hospital, Capital Medical University, Beijing 100045, ChinaHematology Center, National Center for Children's Health, Beijing Children's Hospital, Capital Medical University, Beijing 100045, ChinaHematology Center, National Center for Children's Health, Beijing Children's Hospital, Capital Medical University, Beijing 100045, ChinaLangerhans cell histiocytosis(LCH)and Langerhans cell sarcoma(LCS)are characterized by clone proliferation of Langerhans-type cells, which may occur concurrently or sequentially with T-cell acute lymphoblastic leukemia (T-ALL) and other Lymphoid neoplasms. A 15-year old female patient diagnosed with T-ALL developed LCH involving multiple systems during maintenance chemotherapy of T-AL. After treated with chemotherapy with improved result, the patient showed progression of the illness and refractory to the second-line treatment. We found c.G35A (p.G12D)mutation in the KRAS gene and used the targeted drug Trametinib for treatment. The treatment proved effective, leading to partial remission within a week. Three months after Trametinib treatment, the patient developed new lymphadenopathy. Biopsy revealed the existence of LCS. The disease progressed quickly, and the patient died 7 days after diagnosis of LCS. The case of patients with T-ALL then developing LCH and LCS sequentially is extraordinarily rare. The causes of the case is unclear and may be related to cell transdifferentiation, clonal evolution, and chemotherapy. Targeted drugs can contain this disease for a short time.https://jrd.chard.org.cn/article/doi/10.12376/j.issn.2097-0501.2022.03.013langerhans cell histiocytosislangerhans cell sarcomat-cell acute lymphoblastic leukemiaclonal evolutiontargeted agents |
spellingShingle | TIAN Yu WANG Dong WEI Ang YANG Ying ZHANG Liping MA Honghao WANG Chanjuan CUI Lei LI Zhigang ZHANG Rui WANG Tianyou A Patient with Sequential Diseases of Langerhans Cell Sarcoma, Langerhans Cell Histiocytosis, and Acute Lymphoblastic Leukemia 罕见病研究 langerhans cell histiocytosis langerhans cell sarcoma t-cell acute lymphoblastic leukemia clonal evolution targeted agents |
title | A Patient with Sequential Diseases of Langerhans Cell Sarcoma, Langerhans Cell Histiocytosis, and Acute Lymphoblastic Leukemia |
title_full | A Patient with Sequential Diseases of Langerhans Cell Sarcoma, Langerhans Cell Histiocytosis, and Acute Lymphoblastic Leukemia |
title_fullStr | A Patient with Sequential Diseases of Langerhans Cell Sarcoma, Langerhans Cell Histiocytosis, and Acute Lymphoblastic Leukemia |
title_full_unstemmed | A Patient with Sequential Diseases of Langerhans Cell Sarcoma, Langerhans Cell Histiocytosis, and Acute Lymphoblastic Leukemia |
title_short | A Patient with Sequential Diseases of Langerhans Cell Sarcoma, Langerhans Cell Histiocytosis, and Acute Lymphoblastic Leukemia |
title_sort | patient with sequential diseases of langerhans cell sarcoma langerhans cell histiocytosis and acute lymphoblastic leukemia |
topic | langerhans cell histiocytosis langerhans cell sarcoma t-cell acute lymphoblastic leukemia clonal evolution targeted agents |
url | https://jrd.chard.org.cn/article/doi/10.12376/j.issn.2097-0501.2022.03.013 |
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