The medical recommendations for adult LCH have been published in Orphanet Journal of Rare Diseases.

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Diagnosis

Author(s): Euro Histio Net Work Group for LCH Guidelines for adults (see introduction page), Created: 2012/07/26, last update: 2013/08/18

De­fi­ni­ti­ve dia­gno­sis of LCH re­qui­res clas­si­cal his­topa­tho­lo­gy con­fir­med by de­mons­tra­ti­on of CD1a or Lan­ge­rin (CD207) or by the ul­tra­struc­tu­ral pre­sence of Bir­beck gra­nu­les.

Biopsy and histological examination

The diagnosis of LCH should be based on histologic and immunophenotypic examination of a lesional biopsy. Normal Langerhans cells stain positively with CD1a and/or Langerin. [Lau/Chu/Weiss 2008; Swerdlow et al 2008; Valladeau et al 2000]. Misdiagnoses of LCH have occurred, as the presence of normal reactive LCs in skin and regional lymph nodes may be confusing.

Diagnostic criteria

The two levels of certainty of LCH diagnosis which are generally agreed upon are shown in table 2 [Minkov et al 2009].

Tab. 2.

Diagnostic Criteria of LCH

Diagnosis

Criteria

Definitive
 

= Based on clinic-pathological evidence with microscopic examination and at least one of the following immunological staining:

 
  • Langerin (CD 207) positivity
  • CD1a positivity
  • Presence of Birbeck granules on electronic microscopy

Presumptive
(or compatible)

= Based only on clinico-radiological evidence, without biopsy, as in case of:

 

e.g.: Pulmonary lesions on CT scan with typical cysts and nodules in a smoker (however, biopsy should be considered in order to reach a more definitive diagnosis)

References

[Lau/Chu/Weiss 2008] Lau SK, Chu PG, Weiss LM: Immunohistochemical expression of Langerin in Langerhans cell histiocytosis and non-Langerhans cell histiocytic disorders. The American journal of surgical pathology 2008, 32: 615 [PMID: 18277880]

[Swerdlow et al 2008] Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Thiele J, Vardiman JW: WHO Classification of Tumors of Haematopoietic and Lymphoid Tissues. IARC press, Lyon Fourth Edition. 252, pp.358 [ISBN: 9789283224310

[Valladeau et al 2000] Valladeau J, Ravel O, Dezutter-Dambuyant C, Moore K, Kleijmeer M, Liu Y, Duvert-Frances V, Vincent C, Schmitt D, Davoust J, Caux C, Lebecque S, Saeland S: Langerin, a novel C-type lectin specific to Langerhans cells, is an endocytic receptor that induces the formation of Birbeck granules. Immunity 2000, 12: 71 [PMID: 10661407]

[Minkov et al 2009] Minkov M, Grois N, McClain K, Nanduri V, Rodriguez-Galindo C, Simonitsch-Klupp I, Visser J, Weitzmann S, Whitlock J, Windebank K: Langerhans Cell Histiocytosis - Histiocyte Society Evaluation and Treatment Guidelines. 2009. cited.