Recurrent adult Langerhans cell histiocytosis complicated with diabetes insipidus: report of 1 case and review of literature
10.3760/cma.j.cn115356-20241024-00160
- VernacularTitle:复发成人朗格汉斯细胞组织细胞增生症合并尿崩症1例并文献复习
- Author:
Chen CHEN
1
;
Ruixue LI
;
Yankun YU
;
Weixia NONG
;
Xin PAN
Author Information
1. 石河子大学第一附属医院血液科，石河子 832000
- Keywords:
Langerhans cell histiocytosis;
Diabetes insipidus;
Diagnosis;
Treatment regimen
- From:
Journal of Leukemia & Lymphoma
2025;34(9):548-552
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To improve the understanding of recurrent adult Langerhans cell histiocytosis complicated with diabetes insipidus.Methods:The clinical data of 1 patient with recurrent adult Langerhans cell histiocytosis complicated with diabetes insipidus admitted to the First Affiliated Hospital of Shihezi University in January 2024 was collected. Its disease characteristics, effectiveness and safety of treatment scheme were analyzed, and literatures were reviewed.Results:The 42-year-old female patient was diagnosed as Langerhans cell histiocytosis in June 2021. After treated with cytarabine, the symptoms improved and the patient achieved sustained remission. In January 2024, the patient was admitted to the hospital due to pain in the middle part of the front chest. The PET-CT results indicated disease progression, which was manifested by new bone destruction, enlarged lymph nodes, and increased nocturnal urination, with urine volume of 3-5 L within 24 h. Based on the clinical manifestations such as cranial bone lesions, periorbital soft tissue lesions, and enlarged lymph nodes at onset, multiple systems and multiple foci involvement was considered. The diagnosis of combined diabetes insipidus was confirmed through the water deprivation and pressure test. After MACOP-B regimen (doxorubicin liposome, cyclophosphamide, vincristine, bleomycin, prednisone), the patient's bone pain was completely relieved, and no serious complications occurred.Conclusions:Recurrent adult Langerhans cell histiocytosis complicated with diabetes insipidus is rare; MACOP-B regimen is safe and effective in treatment of the disease.
