Patients with diabetes insipidus and/or skull lesions in the orbit, mastoid or temporal bones appear to be at higher risk for Langerhans cell histiocytosis (LCH) central nervous system (CNS) involvement and LCH CNS neurodegenerative syndrome. These patients should have magnetic resonance imaging (MRI) scans with gadolinium contrast at the time of LCH diagnosis and every 2 years thereafter for 10 years to detect evidence of CNS disease. The Histiocyte Society CNS LCH Committee does not recommend any treatment for radiologic CNS LCH of the neurodegenerative type. However, being aware of its presence is important and careful neurologic examinations and appropriate imaging with MRIs are done at regular intervals. Brain stem auditory evoked responses should also be done at regular intervals to define the onset of clinical CNS LCH. When clinical signs are present, intervention may be indicated.

Vertebral lesions can be effectively followed by MRI scans and pelvic lesions by computed tomography (CT) scans or MRI scans.

For children with LCH in the lung, pulmonary function testing and chest CT scans are sensitive methods for detecting disease progression.[1]

References

1. Bernstrand C, Cederlund K, Henter JI: Pulmonary function testing and pulmonary Langerhans cell histiocytosis. Pediatr Blood Cancer 49 (3): 323-8, 2007.  [PUBMED Abstract]
   
   

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