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Home > West Medicine > Pediatrics > Pediatric Internal Med
D47.700x004

Langerhans-Cell Histiocytosis

儿科综合

Common symptoms

肝功能异常、斑丘疹、肝脾肿大、腹泻、共济失调、腹水、肝纤维化、骨溶解、肝功能衰竭、构音障碍

The pathology of disease

Langerhans-cell histiocytosis (LCH), formerly called histiocytosis, is a group of idiopathic histiocyte-proliferative disorders. Traditionally divided into three clinical types: Letterer-Siwe disease (L-S), Hand-Schuller-Christian disease (H-S-C), and eosinophilic granuloma of bone (EGB). Cause is unknown; recent research links it to in-vivo immune dysregulation.

Common Symptoms

Abnormal liver function; maculopapular rash; hepatosplenomegaly; diarrhea; ataxia; ascites; hepatic fibrosis; osteolysis; liver failure; dysarthria

Causes and Pathogenesis

Etiology unclear; although genetic features are unknown, it has some familial tendency, with much higher incidence among siblings than ordinary children; some consider it neoplastic in nature.

Treatment

Surgery, radiotherapy, drug therapy, supportive therapy

Preventive Measures

In systemic LCH patients, monitor for chronic disability from disease/treatment - cosmetic/functional orthopedic issues, skin lesions and neurotoxicity, as well as emotional fluctuations.

Department to Visit

It is recommended to visit the General Pediatrics department; an appointment can be booked on this platform before the visit.

Warm reminder: The above information is for health science reference only and cannot replace professional medical diagnosis. If you have related symptoms, please seek medical attention at a regular hospital promptly.