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.^[1]^[2]^[3]^[4]^[5]^[6]

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.^[3]

Complications

Common complications of Langerhans cell histiocytosis include:^[1]^[2]^[3]^[4]^[5]^[6]

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.^[1]^[2]^[3]^[4]^[5]^[6]
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.

References

↑ ^1.0 ^1.1 ^1.2 Komp DM, El Mahdi A, Starling KA, Easley J, Vietti TJ, Berry DH; et al. (1980). "Quality of survival in histiocytosis X: a Southwest Oncology Group study". Med Pediatr Oncol. 8 (1): 35–40. PMID 6969347.
↑ ^2.0 ^2.1 ^2.2 Langerhans Cell Histiocytosis Treatment (PDQ®): Health Professional Version. National Cancer Institute (2015) http://www.cancer.gov/types/langerhans/hp/langerhans-treatment-pdq Accessed on February, 3 2016
↑ ^3.0 ^3.1 ^3.2 ^3.3 DiCaprio MR, Roberts TT (2014). "Diagnosis and Management of Langerhans Cell Histiocytosis". J Am Acad Orthop Surg. 22 (10): 643–652. doi:10.5435/JAAOS-22-10-643. PMID 25281259.
↑ ^4.0 ^4.1 ^4.2 Harmon CM, Brown N (2015). "Langerhans Cell Histiocytosis: A Clinicopathologic Review and Molecular Pathogenetic Update". Arch Pathol Lab Med. 139 (10): 1211–4. doi:10.5858/arpa.2015-0199-RA. PMID 26414464.
↑ ^5.0 ^5.1 ^5.2 Langerhans cell histiocytosis. Wikipedia (2015) https://en.wikipedia.org/wiki/Langerhans_cell_histiocytosis Accessed on February, 2 2016
↑ ^6.0 ^6.1 ^6.2 Langerhans cell histiocytosis. Radiopeadia (2015) http://radiopaedia.org/articles/langerhans-cell-histiocytosis Accessed on February, 3 2016

Template:WikiDoc Sources

[pmid6969347-1] 1.0 ^1.1 ^1.2 Komp DM, El Mahdi A, Starling KA, Easley J, Vietti TJ, Berry DH; et al. (1980). "Quality of survival in histiocytosis X: a Southwest Oncology Group study". Med Pediatr Oncol. 8 (1): 35–40. PMID 6969347.

[PDQ-2] 2.0 ^2.1 ^2.2 Langerhans Cell Histiocytosis Treatment (PDQ®): Health Professional Version. National Cancer Institute (2015) http://www.cancer.gov/types/langerhans/hp/langerhans-treatment-pdq Accessed on February, 3 2016

[pmid25281259-3] 3.0 ^3.1 ^3.2 ^3.3 DiCaprio MR, Roberts TT (2014). "Diagnosis and Management of Langerhans Cell Histiocytosis". J Am Acad Orthop Surg. 22 (10): 643–652. doi:10.5435/JAAOS-22-10-643. PMID 25281259.

[pmid26414464-4] 4.0 ^4.1 ^4.2 Harmon CM, Brown N (2015). "Langerhans Cell Histiocytosis: A Clinicopathologic Review and Molecular Pathogenetic Update". Arch Pathol Lab Med. 139 (10): 1211–4. doi:10.5858/arpa.2015-0199-RA. PMID 26414464.

[wiki-5] 5.0 ^5.1 ^5.2 Langerhans cell histiocytosis. Wikipedia (2015) https://en.wikipedia.org/wiki/Langerhans_cell_histiocytosis Accessed on February, 2 2016

[radio-6] 6.0 ^6.1 ^6.2 Langerhans cell histiocytosis. Radiopeadia (2015) http://radiopaedia.org/articles/langerhans-cell-histiocytosis Accessed on February, 3 2016

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