What is the prognosis of Langerhans cell histiocytosis (LCH)?

Updated: Sep 16, 2020
  • Author: Cameron K Tebbi, MD; Chief Editor: Vikramjit S Kanwar, MBBS, MBA, MRCP(UK), FAAP  more...
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Answer

The location of the lesions and the extent of the disease substantially affect the course of the disease and the patient’s prognosis.

Involvement of risk organs (hemopoietic system, liver, spleen, and lungs) at diagnosis and failure of response to the initial therapy are poor prognostic signs. Reactivation of risk organs is relatively rare. In one study, this reactivation occurred in 2% of patients. [330] Involvement of the risk organs at reactivation had relatively low impact on survival. The degree of organ involvement is correlated with the patient’s prognosis.

Although Langerhans cell histiocytosis involvement of the spine causes lesions and, sometimes, asymmetric collapse, it is not usually associated with long-term sequelae and deformity. Therefore, aggressive surgical management of this involvement is generally not indicated.

Rapidity of the response to chemotherapy may also have prognostic value.

A study by Zeng et al suggested that in LCH, the presence of BRAF-V600E mutation is related to poor disease-free survival, with the mutation leading to interference with host-tumor immune surveillance. [331]


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