The Clinical Analysis Of Three Cases Of Langerhans Cell Histiocytosis With Literature Review

Background and Objective Langerhans cell histiocytosis (LCH) is a proliferative disease of immature dendritic cell (DC). The pathogenesis of LCH remains obscure. Any system might be involvedt with variable manifestations. Because of its low incidence and mostly in children, the definitive treatment strategy remains vague for adult patients. Along with the report of 3 cases treated in our hospital with cytarabine, we carried out a review of the clinical characteristics, prognosis and treatment options, aiming to present data to clinicians and relevant patients.Methods Clinical data of 3 adult LCH cases were retrospectively analyzed. All of the patients were diagnosed and treated in Hematology and oncology unit of geriatrics department, Qilu Hospital of Shandong University. Case reports of adult are retrieved from CNKI, WanFang and pubMed of Chinese patients from 2010 to 2015 and 66 cases being found with comparatively well data. Combining these cases with the above 3 cases,69 cases were analyzed. The general information such as sex and age, clinical manifestations, treatment and prognosis of LCH are analyzed.Results The three patients diagnosed and treated in our department we report were all multi-system LCH (MS-LCH), including 2 cases of recurrent disease and 2 patients of high risk. After treatment with cytarabine (Ara-C), the 2 recurrent diseases achieved effect of no active disease (NAD) for at least one year. The last patient’s hepatic lesions had progression and got improvement after oral etoposide. For the 69 patients, male 39 cases, female30 cases, with median age of 36 years old and average age of 38.1 years old. SS-LCH 37 cases, MS-LCH 32 cases. The involved organs were bone 31 (23.9%), lung 28 (21.5%), lymph nodes 15 (11.6%), pituitary-hypothalamus 13 (10.0%), mucocutaneous 10 (7.8%), female genitourinary system 11 (8.6%), liver 9 (6.9%), thyroid gland 5 (3.9%), bone marrow and glandula angularis 2 (1.5%), kidney, sublingual gland, spleen 1 (0.7%). SS-LCH were treated with surgery, radiotherapy or chemotherapy, while MS-LCH mostly treated with systemic chemotherapy. The prognosis of SS-LCH was better than MS-LCH.Conclusion LCH is a group of disease characterized by monoclonal accumulation of langerhans cells in different organs. Its clinical symptom is diversiform, and it can be diagnosed in different departments. The histopathology and immunohistochemical examination are critical. As the organs involvement and manifestion are different, the treatment and prognosis are not same. Ara-C was the first drug combined with vincristine and prednisone in multi-organ patients, with good curative effect observed. In order to improve the survival rate in adult patients, prospective clinical study and etiology research is necessary.