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]

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.

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%.

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