Table of contents
Langerhans cell histiocytosis
What's new
Added 2022 HS guidelines for the diagnosis and management of Langerhans cell histiocytosis.
Guidelines
Key sources
The following summarized guidelines for the evaluation and management of langerhans cell histiocytosis are prepared by our editorial team based on guidelines from the Histiocyte Society (HS 2022)....
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Screening and diagnosis
Diagnostic investigations
Laboratory testing
As per HS 2022 guidelines:
Obtain laboratory tests to assess for liver insufficiency, cytopenias, markers of inflammation (CRP) at diagnosis.
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Obtain laboratory tests to rule out diabetes insipidus and anterior pituitary function in patients with polyuria/polydipsia or involvement of pituitary/hypothalamus axis on cranial imaging.
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Diagnostic imaging
Pulmonary function testing
Echocardiography
Right heart catheterization
Cholangiopancreatography
Diagnostic procedures
Biopsy: as per HS 2022 guidelines, Perform a biopsy of lesional tissue even in circumstances of highly suggestive clinical and imaging features to confirm LCH diagnosis and to establish BRAF or other MAPK-ERK pathway mutational status.
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Mutational analysis
Medical management
Management of unifocal LCH: as per HS 2022 guidelines, Offer observation or local therapies, such as surgical excision, intralesional corticosteroids, or radiation, as first-line treatments in patients with unifocal LCH (except diabetes insipidus).
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Management of pulmonary LCH
Management of multifocal LCH
Follow-up and surveillance
Assessment of treatment response
As per HS 2022 guidelines:
Decide on the type and frequency of response assessments and follow-up examinations depending on the degree of involvement with LCH.
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Obtain repeated FDG-FDG PET-based imaging study for assessment of disease response after 2-3 months of initiation of therapy in patients with initially FDG-FDG PET-avid LCH. Decide on subsequent imaging frequency individually based on specific clinical scenario.
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