A Patient with Sequential Diseases of Langerhans Cell Sarcoma, Langerhans Cell Histiocytosis, and Acute Lymphoblastic Leukemia
10.12376/j.issn.2097-0501.2022.03.013
- VernacularTitle:相继发生急性T淋巴细胞白血病、朗格汉斯细胞组织细胞增生症和朗格汉斯细胞肉瘤一例
- Author:
Yu TIAN
1
;
Dong WANG
2
;
Ang WEI
2
;
Ying YANG
2
;
Liping ZHANG
2
;
Honghao MA
2
;
Chanjuan WANG
2
;
Lei CUI
2
;
Zhigang LI
2
;
Rui ZHANG
2
;
Tianyou WANG
2
Author Information
1. Hematology Center, National Center for Children's Health, Beijing Children's Hospital, Capital Medical University, Beijing 100045, China.Department of Child Health Care, Affiliated Children's Hospital of Capital Institute of Pediatrics, Beijing 100020, China.
2. Hematology Center, National Center for Children's Health, Beijing Children's Hospital, Capital Medical University, Beijing 100045, China.
- Publication Type:Journal Article
- Keywords:
Langerhans cell histiocytosis;
Langerhans cell sarcoma;
T-cell acute lymphoblastic leukemia;
clonal evolution;
targeted agents
- From:
JOURNAL OF RARE DISEASES
2022;1(3):311-317
- CountryChina
- Language:Chinese
-
Abstract:
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.
