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» Expert Histio Net » For Professionals » Guidelines » LCH adult » Treatment » Reactivation - treatment options  · 

Treatment options in case of reactivation

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

Reactivations of LCH in adults occur in about 25-38% of the patients (European national registry data and [Cantu et al 2012]). Patients may have further reactivations especially those with multisystem disease.

Reactivation of single system disease

The choice of treatment options is based on the same principles as for initial disease.

The options for reactivations of SS-LCH (skin, bone, other) include

Wait and watch approach
Local therapy including irradiation (see local therapy)
Bisphosphonates for bony disease
Chemotherapy (see systemic therapy)

In case of a multisystem reactivation of a SS-LCH, treatment should follow the options for MS-LCH including systemic therapy. (Grade D2 *)

The efficacy of 2-CDA for single and multisystem reactivated LCH has been proved in a phase II trial [Saven/Burian 1999].

Reactivation after systemic therapy

  • If the reactivation is more than one year after completion of treatment, re-induction with the prior chemotherapy may be effective. If however, the disease is not responsive we suggest discussion with the reference centre for your country.
  • If reactivation occurs while on treatment, potentially 2nd line strategies as described (see salvage therapy), but should be generally discussed with your reference centre. (Grade D2 *)

* Grades of recommendation see here.


[Cantu et al 2012] Cantu MA, Lupo PJ, Bilgi M, Hicks MJ, Allen CE, McClain KL: Optimal therapy for adults with Langerhans cell histiocytosis bone lesions. PloS one 2012, 7:e43257 [PMID: 22916233]

[Saven/Burian 1999] Saven A, Burian C: Cladribine activity in adult langerhans-cell histiocytosis. Blood 1999 Jun 15; 93: 4125 [PMID: 10361109]