The medical guidelines for childhood LCH have been published in Pediatric Blood & Cancer  (PBC), Wiley Online Library.

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Diagnostic Criteria

Author(s): Euro Histio Net Work Group for LCH Guidelines (see introduction page), Created: 2011/03/09, last update: 2012/01/23

The diagnosis is clinicopathologic and should only be made in the correct clinical setting to prevent misdiagnosis in the presence of normal reactive Langerhans cells particularly in regional lymph nodes.

The two levels of certainty of LCH diagnosis which are generally agreed upon are shown in the following Table 2.

Tab. 2.

Diagnostic Criteria of LCH

Diagnosis

Criteria

Definitive
 

= Based on 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:

 
  • Typical bone lesion (in a difficult to reach region) with/without diabetes insipidus
  • 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)