Home > D. Systemic pathology > Hematopathology > Langerhans cell histiocytosis

Langerhans cell histiocytosis

Friday 24 October 2003

Digital cases

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

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

Portfolio

  • Cutaneous Langerhans histiocytosis
  • Cutaneous Langerhans histiocytosis
  • Cutaneous Langerhans histiocytosis
  • Birbeck granule
  • Birbeck granules
  • Birbeck granules
  • S-100 immunostaining
  • CD1a immunostaining in Langerhans histiocytosis
  • Femoral Langerhans histiocytosis
  • Femoral Langerhans histiocytosis