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

Objective Langerhans cell histiocytosis is a rare proliferative disease of pathological Langerhans cells. Its pathogenesis is unclear and the biological behavior is diverse. Currently cases reported are mainly in children.The low incidence of adult LCH and few clinical reports result in our lack of knowledge of the disease.The aim of the following paper is to summarize the clinical features, diagnosis and treatment of adult LCH patients, and to raise awareness of the disease and improve the diagnosis and treatment.Method Report the clinical data of4cases adult LCH confirmed by pathology oftissue hyperplasia. Retrieve case reports of adult LCH published on Chinese Journal such as CNKI and WanFang in recent years, Of62cases of patients which were comparatively well data, combined with the above4cases,66cases were analyzed. Summary the pathogenesis, diagnostic criteria, treatment and prognosis of LCH with review of the literature.Results The four patients we report were adult-onset, including3cases of multi-system disease, diagnosed by lymph node biopsy. After giving different courses of chemotherapy, all of3patients achieved partial remission, their clinical symptoms or performance imaging improved. The other patient was a single focal single system whose main symptom was bone destruction. After the surgical removal of the lesion and chemotherapy,1year follow-up condition has stabilized. For the66patients, male39cases, female27cases, with an average age of37.5years old. SS-LCH14cases, MS-LCH52cases. The involved organs were bone28(42.4%), lymph nodes28(42.4%), skin24(36.4%), lung22(28.8%), pituitary19 (33.3%), soft tissue12(18.2%), liver and spleen17(25.8%), bone marrow7(10.6%), thyroid gland7(10.6%), eye6(9%), ear3(4.5%), submandibular gland1(1.5%), kidney1(1.5%). SS-LCH were given more simple surgery, hormones or radiotherapy, MS-LCH were given priority to systemic chemotherapy. The efficacy and prognosis of SS-LCH were better than MS-LCH.Conclusion Langerhans cell histiocytosis is a rare histiocytic disorder, the pathologic basis of which is a single-macrophage system with Langerhans cells. The clinical manifestations of LCH is diversiform, and the misdiagnosis rate is high. Its diagnosis mainly relies on histopathology and immunohistochemical examination. As the age, degree of cellpro-liferation, the tissues and organs involved are different, the symptoms and prognosis are not the same.