Langerhans cell histiocytosis natural history

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Haytham Allaham, M.D. [2]

Overview

The clinical course of Langerhans cell histiocytosis may range from a unifocal disease that spontaneously regress to a rapidly progressive disease with multi-system involvement.

Depending on the extent of the tumor at the time of diagnosis, the prognosis may vary. However, the prognosis is generally regarded as excellent.
The 5-year survival rate of patients with unifocal Langerhans cell histiocytosis is approximately 99%.
The 5-year survival rate of patients with multifocal unisystem Langerhans cell histiocytosis is approximately 97%.
The 5-year survival rate of patients with multifocal multisystem Langerhans cell histiocytosis (with risk of organ failure) is approximately 77%.
The table below lists prognostic factors for Langerhans cell histiocytosis patients:


Prognostic Factor	Description

Age	A younger age at the time of diagnosis is associated with a worse prognosis.
Organ involvement	A more extensive organ involvement is associated with a worse prognosis.
Response to treatment	A modest response rate after 6 weeks of therapy is associated with a worse prognosis.
Cellular markers	The expression of metalloproteinase and gelosin on a cellular level is associated with a worse prognosis.