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Langerhans cell histiocytosis

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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Guidelines

1.Screening and diagnosis

Diagnosis: suspect LCH in the presence of characteristic clinical/radiologic features, even when histopathologic review is equivocal.
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2.Diagnostic investigations

Laboratory testing
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

3.Diagnostic procedures

Biopsy: perform a biopsy of lesional tissue even in circumstances of highly suggestive clinical and imaging features to confirm LCH diagnosis and to establishBRAF or other MAPK-ERK pathway mutational status.
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  • Mutational analysis

4.Medical management

Management of unifocal LCH: 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

5.Follow-up and surveillance

Assessment of treatment response
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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