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. Common complications of Langerhans cell histiocytosis include pulmonary fibrosis, pathological bone fractures, and central diabetes insipidus. Depending on the extent of the tumor at the time of diagnosis, the prognosis may vary. However, the prognosis is generally regarded as excellent.

Natural History

• 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.
• Pulmonary Langerhans cell histiocytosis generally exhibits an insidious and progressive disease pattern that makes it a diagnostic challenge to catch the disease at an early disease phase. The mean age of the patients at the time of presentation, therefore, may not reflect the true age of disease onset. Pulmonary LCH typicaly presents as bronchiolitis that may progress to intraluminal fibrosis and elastic fiber degradation and lung remodeling.

Complications

• Common complications of Langerhans cell histiocytosis include:

• Pulmonary fibrosis
• Pulmonary hypertension
• Pathological bone fractures
• Anemia
• Recurrent bleeding
• Recurrent infections
• Central diabetes insipidus

Prognosis

• 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.

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