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Langerhans Cell Histiocytosis Treatment (PDQ®)

Health Professional Version

Follow-up Considerations in Childhood LCH

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 1 to 2 years thereafter for 10 years to detect evidence of CNS disease.[1] The Histiocyte Society CNS LCH Committee does not recommend any treatment for radiologic CNS LCH of the neurodegenerative type if there is no associated clinical neurodegeneration. However, careful neurologic examinations and appropriate imaging with MRI is suggested at regular intervals. Brain stem auditory evoked responses should also be done at regular intervals to define the onset of clinical CNS LCH as early as possible, as this may affect response to therapy.[2] When clinical signs are present, intervention may be indicated. Available studies of different forms of therapy for CNS neurodegeneration suggest that the neurodegenerative changes may be stabilized or improved, but only if therapy is started early.[2] (Refer to the LCH CNS neurodegenerative syndrome section of this summary for more information.) Careful follow-up of patients at risk is critical.

For children with LCH in the lung, pulmonary function testing and chest computed tomography scans are sensitive methods for detecting disease progression.[3]

In summary, many patients with multisystem disease will experience long-term sequelae due to their underlying disease and/or treatment. Endocrine and CNS sequelae are the most common. These long-term sequelae significantly affect health quality of life in many of these patients.[4][Level of evidence: 3iiiC] Specific long-term follow-up guidelines after treatment of childhood cancer or in those who have received chemotherapy have been published by the Children's Oncology Group and are available on their Web site.

References

  1. Wnorowski M, Prosch H, Prayer D, et al.: Pattern and course of neurodegeneration in Langerhans cell histiocytosis. J Pediatr 153 (1): 127-32, 2008. [PUBMED Abstract]
  2. Allen CE, Flores R, Rauch R, et al.: Neurodegenerative central nervous system Langerhans cell histiocytosis and coincident hydrocephalus treated with vincristine/cytosine arabinoside. Pediatr Blood Cancer 54 (3): 416-23, 2010. [PUBMED Abstract]
  3. Bernstrand C, Cederlund K, Henter JI: Pulmonary function testing and pulmonary Langerhans cell histiocytosis. Pediatr Blood Cancer 49 (3): 323-8, 2007. [PUBMED Abstract]
  4. Nanduri VR, Pritchard J, Levitt G, et al.: Long term morbidity and health related quality of life after multi-system Langerhans cell histiocytosis. Eur J Cancer 42 (15): 2563-9, 2006. [PUBMED Abstract]
  • Updated: June 4, 2014