Langerhans cell histiocytosis (LCH): investigations

Written and reviewed by Dr Ibrahim Imam, Consultant Neurologist. Last reviewed .

Langerhans cell histiocytosis (LCH): investigations. The histiocytic cells originate from bone marrow-derived immature myeloid dendritic cells.

Pathological features

  • The histiocytic cells originate from bone marrow-derived immature myeloid dendritic cells
  • They are S‐100, CD1a, and CD207 (Langerin) positive cells
  • They have abundant eosinophilic cytoplasm
  • There appear as Birbeck granules on electron microscopy:

Haematological tests

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Endocrine tests

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Pulmonary tests

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Cardiac tests

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Gastrointestinal and hepatobiliary tests

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Molecular analysis

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Magnetic resonance imaging (MRI) brain: features

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Other imaging tests

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Other tests

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Synonym

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References

  1. Satter EK, High WA. Langerhans cell histiocytosis: a review of the current recommendations of the Histiocyte Society. Pediatr Dermatol 2008; 25:291-295.
  2. Leung AKC, Lam JM, Leong KF. Childhood Langerhans cell histiocytosis: a disease with many faces. World J Pediatr 2019; 15:536-545.
  3. Girschikofsky M, Arico M, Castillo D, et al. Management of adult patients with Langerhans cell histiocytosis: recommendations from an expert panel on behalf of Euro-Histio-Net.Orphanet J Rare Dis 2013; 8:72.
  4. Harmon CM, Brown N. Langerhans cell histiocytosis: a clinicopathologic review and molecular pathogenetic update. Arch Pathol Lab Med 2015; 139:1211-1214.
  5. Goyal G, Tazi A, Go RS, et al. International expert consensus recommendations for the diagnosis and treatment of Langerhans cell histiocytosis in adults. Blood 2022; 139:2601-2621.

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