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» Expert Histio Net » For Professionals » Guidelines » LCH childhood » Treatment » Treatment of Reactivations  · 

Treatment Options in Case of Reacitvation

Author(s): Euro Histio Net Work Group for LCH Guidelines (see introduction page), Created: 2011/05/20, last update: 2014/02/09

Whenever possible patients should be enrolled in ongoing clinical trials and treated according to the protocol. CHILDREN who are not enrolled in a clinical trial should be treated according to the guidelines presented here. Due to the diversity of clinical course of LCH, even recommendations which are established as standard of care may need to be critically appraised in an individual case. We suggest that you never hesitate to contact LCH experts in case that the clinical course raises questions or doubts.

Reactivation of Single System Langerhans Cell Histiocytosis (SS-LCH)

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

The options for reactivations of single system LCH (skin, bone, other) include:
I. Wait and watch approach
II. Local therapy (details see here)
III. Indomethacin for bony disease
IV. Vinblastine (VBL) + steroid (details see here)

Radiotherapy is no longer recommended due to the long-term sequelae.

In case of a multisystemic reactivation of a SS-LCH, treatment should follow the options for MS-LCH including systemic therapy.

Whenever possible patients should be enrolled in ongoing clinical trials and treated according to the protocol. CHILDREN who are not enrolled in a clinical trial should be treated according to the guidelines presented here. Due to the diversity of clinical course of LCH, even recommendations which are established as standard of care may need to be critically appraised in an individual case. We suggest that you never hesitate to contact LCH experts in case that the clinical course raises questions or doubts.

Reactivation after Systemic Therapy

  • If the reactivation is after completion of treatment, re-induction with VBL + steroid may be effective and there may be no need to switch to alternative therapy. If however, the disease is not responsive we suggest discussion with the reference centre for your country.
  • If reactivation occurs while on treatment, 2nd line strategies should be discussed with your reference centre, taking into account disease severity and the respective risk for unfavorable outcome.