Multisystem Langerhans cell histiocytosis with diabetes insipidus as the first symptom: a case report and literature review
10.3760/cma.j.issn.1009-9921.2014.12.011
- VernacularTitle:以尿崩症为首发症状的多系统郎格汉斯细胞组织细胞增生症一例并文献复习
- Author:
Luxi SONG
;
Cha GUO
;
Yao ZHANG
;
Shuang HAN
;
Qingxia ZHANG
;
Jin HUANG
;
Chunkang CHANG
- Publication Type:Journal Article
- Keywords:
Histiocytosis,Langherhans-cell;
Diabetes insipidus;
Diagnosis
- From:
Journal of Leukemia & Lymphoma
2014;23(12):743-745
- CountryChina
- Language:Chinese
-
Abstract:
Objective To study the clinical features and differential diagnosis of Langerhans cell histiocytosis (LCH).Methods A case of LCH was reported and the literatures were reviewed.Results The of multisystem LCH patient,presented with a diabetes insipidus (DI) and panhypopituitarism,was 44 years old,and developed costal,tibial and femoral multiple lesions.The final diagnosis as LCH was made based on biopsy of tibia and lymph nodes.The biopsy specimen showed that the cells were infiltrated exhibiting the characteristic morphologic features of Langerhans cell (LC) with a convoluted shape,elongated nuclei exhibiting longitudinal grooves,and immunohistochemistry results revealed positive LC for the S-100,CD1a and Langerin immunostaining.Conclusions LCH may range from a solitary lytic bone lesion (for example eosinophilic granuloma) with a favorable course to a fatal disseminated leukaemia-like form.LCH typically involves the bone,lesions almost can be found in all organs.DI and CNS involvement often present as a puzzling syndrome,which renders the diagnosis problematicly,and often delays the diagnosis of LCH.The damage to the pituitary/hypothalamus axis results in life-long hormonal replacement therapy.
