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Home > West Medicine > ENT & Ophthalmology > Ophthalmology
C96.000

Letterer-Siwe Disease

眼科

Common symptoms

丘疹、淋巴结肿大、呼吸困难、腹泻、结节、持续性发热、血小板减少、眼睑闭合不全、囊肿、脓肿

The pathology of disease

Letterer-Siwe disease is an acute differentiated histiocytosis; young age at onset; lesions often involve multiple systems; eye/orbital involvement rare; very poor prognosis. It is a subtype of Langerhans-cell histiocytosis.

Common Symptoms

Papule; lymph-node enlargement; dyspnea; diarrhea; nodule; persistent fever; thrombocytopenia; lagophthalmos; cyst; abscess

Causes and Pathogenesis

Most view it as a differentiated histiocytosis between immune-reactive non-neoplastic proliferation and malignant histiocytosis; some view it as a malignant clonal disease. Most studies place it between benign and malignant tumors.

Treatment

Drug, supportive therapy

Preventive Measures

No effective prevention; early diagnosis/treatment.

Department to Visit

It is recommended to visit the Ophthalmology 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.