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Langerhans cell histiocytosis

Friday 24 October 2003

Definition : Langerhans cell histiocytosis (histiocytosis X) is a disorder of unknown etiology characterized by solitary (eosinophilic granuloma) or multiple bone and visceral masses.

Cytologically, the smears are hypercellular with numerous Langerhans cell histiocytes, which are large, polygonal cells with eosinophilic cytoplasm and oval nuclei with longitudinal grooves (‘coffee bean nuclei’).

Scattered eosinophils, mixed inflammatory cells, osteoclast-like giant cells, and necrosis are identified in the background.

The histiocytes stain with CD1a, S100, vimentin, and CD68 (variable), and are negative for HAM56, CD21, and CD35.

Tennis racket-shaped, electron-dense cross striations (Birbeck granules) are identified on electron microscopy.

While the presence of eosinophils may be a clue to the diagnosis, the distinctive immunohistochemical pattern of the histiocytes differentiates Langerhans cell histiocytosis from XG disease.

Digital cases

- HPC:245 : Orbital Langerhans cell histiocytosis
- JRC:1610 : Pulmonary localized Langerhans cell histiocytosis.
- JRC:6344 : Pulmonary localized Langerhans cell histiocytosis.

Images

- https://twitter.com/CArnold_GI/status/726767164242419712

Epidemiology

- congenital Langerhans cell histiocytosis (rare)

Ultrastructure

- Birbeck granules

Localization

- skin
- digestive system
- lymph node
- liver
- female genital tract (12801274)

Variants

- malignant Langerhans cell tumor (11437944, 12963924)

Immunochemistry

- CD1a+
- Langherin +
- S100+
- CD68-
- fascine+

CGH

- losses of DNA sequences on chromosomes 1p, 5, 6, 7, 9, 16, 17, and 22q (12094383)

- gain of DNA copy number was seen on chromosomes 2q, 4q, and 12 (12094383)

LOH (17378622)

- 1p LOH (12094383)
- 5q23 LOH (17378622)
- Chr.7 LOH (12094383)
- Chr.9 LOH (12094383)
- 22q LOH (12094383)

Molecular biology

- Detection of clonal lymphoid receptor gene rearrangements in langerhans cell histiocytosis. (20551822)

Case records

- Case 11335: Femoral Langerhans histiocytosis
- Case 12691: Faciocranial Langerhans cell histiocytosis

References

- Detection of clonal lymphoid receptor gene rearrangements in langerhans cell histiocytosis. Chen W, Wang J, Wang E, Lu Y, Lau SK, Weiss LM, Huang Q. Am J Surg Pathol. 2010 Jul;34(7):1049-57. PMID: 20551822

- Chikwava KR, Hunt JL, Mantha GS, Murphy JE, Jaffe R. Analysis of loss of heterozygosity in single-system and multisystem langerhans’ cell histiocytosis. Pediatr Dev Pathol. 2007 Jan-Feb;10(1):18-24. PMID: 17378622

- Murakami I, Gogusev J, Fournet JC, Glorion C, Jaubert F. Detection of molecular cytogenetic aberrations in langerhans cell histiocytosis of bone. Hum Pathol. 2002 May;33(5):555-60. PMID: 12094383

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