Langerhans Cell Histiocytosis Treatment - Childhood LCH
Prognosis and treatment are closely linked to the extent of disease at presentation and whether high-risk organs (liver, spleen, lung, and/or bone marrow) are involved. Patients with single-system disease and low-risk multisystem disease do not usually die from LCH, but recurrent disease may result in considerable morbidity and significant late effects. Involvement of craniofacial bones including orbital, mastoid, and temporal bones is associated with an increased risk of diabetes insipidus in addition to increased frequency of anterior pituitary hormone deficiencies and neurologic problems. Although age younger than 2 years was once thought to portend a worse prognosis, data from the LCH-II study showed that patients aged 2 years or younger without high-risk organ involvement had the same response to therapy as older patients. By contrast, the overall survival (OS) was poorer in neonates with risk-organ involvement compared with infants and children with the same extent of disease. In general, however, response to therapy at 6 to 12 weeks has been shown to be more important than age. The overall response to therapy is influenced by the duration and intensity of treatment.[1,2]
(Refer to the Endocrine system subsection in the Multisystem Disease Presentation section of this summary for more information on diabetes insipidus.)
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