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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 fracture]]s, 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.
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 fracture]]s, 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==
==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.<ref name="pmid25281259">{{cite journal| author=DiCaprio MR, Roberts TT| title=Diagnosis and Management of Langerhans Cell Histiocytosis. | journal=J Am Acad Orthop Surg | year= 2014 | volume= 22 | issue= 10 | pages= 643-652 | pmid=25281259 | doi=10.5435/JAAOS-22-10-643 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25281259  }} </ref>
* 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.<ref name="pmid25281259">{{cite journal| author=DiCaprio MR, Roberts TT| title=Diagnosis and Management of Langerhans Cell Histiocytosis. | journal=J Am Acad Orthop Surg | year= 2014 | volume= 22 | issue= 10 | pages= 643-652 | pmid=25281259 | doi=10.5435/JAAOS-22-10-643 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25281259  }} </ref>
* 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.
 
==Complications==
==Complications==
* Common [[complications]] of Langerhans cell histiocytosis include:<ref name="pmid6969347">{{cite journal| author=Komp DM, El Mahdi A, Starling KA, Easley J, Vietti TJ, Berry DH et al.| title=Quality of survival in histiocytosis X: a Southwest Oncology Group study. | journal=Med Pediatr Oncol | year= 1980 | volume= 8 | issue= 1 | pages= 35-40 | pmid=6969347 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6969347  }} </ref><ref name="PDQ">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</ref><ref name="pmid25281259">{{cite journal| author=DiCaprio MR, Roberts TT| title=Diagnosis and Management of Langerhans Cell Histiocytosis. | journal=J Am Acad Orthop Surg | year= 2014 | volume= 22 | issue= 10 | pages= 643-652 | pmid=25281259 | doi=10.5435/JAAOS-22-10-643 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25281259  }} </ref><ref name="pmid26414464">{{cite journal| author=Harmon CM, Brown N| title=Langerhans Cell Histiocytosis: A Clinicopathologic Review and Molecular Pathogenetic Update. | journal=Arch Pathol Lab Med | year= 2015 | volume= 139 | issue= 10 | pages= 1211-4 | pmid=26414464 | doi=10.5858/arpa.2015-0199-RA | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26414464  }} </ref><ref name="wiki">Langerhans cell histiocytosis. Wikipedia (2015) https://en.wikipedia.org/wiki/Langerhans_cell_histiocytosis Accessed on February, 2 2016</ref><ref name="radio">Langerhans cell histiocytosis. Radiopeadia (2015) http://radiopaedia.org/articles/langerhans-cell-histiocytosis Accessed on February, 3 2016</ref>
* Common [[complications]] of Langerhans cell histiocytosis include:<ref name="pmid6969347">{{cite journal| author=Komp DM, El Mahdi A, Starling KA, Easley J, Vietti TJ, Berry DH et al.| title=Quality of survival in histiocytosis X: a Southwest Oncology Group study. | journal=Med Pediatr Oncol | year= 1980 | volume= 8 | issue= 1 | pages= 35-40 | pmid=6969347 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6969347  }} </ref><ref name="PDQ">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</ref><ref name="pmid25281259">{{cite journal| author=DiCaprio MR, Roberts TT| title=Diagnosis and Management of Langerhans Cell Histiocytosis. | journal=J Am Acad Orthop Surg | year= 2014 | volume= 22 | issue= 10 | pages= 643-652 | pmid=25281259 | doi=10.5435/JAAOS-22-10-643 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25281259  }} </ref><ref name="pmid26414464">{{cite journal| author=Harmon CM, Brown N| title=Langerhans Cell Histiocytosis: A Clinicopathologic Review and Molecular Pathogenetic Update. | journal=Arch Pathol Lab Med | year= 2015 | volume= 139 | issue= 10 | pages= 1211-4 | pmid=26414464 | doi=10.5858/arpa.2015-0199-RA | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26414464  }} </ref><ref name="wiki">Langerhans cell histiocytosis. Wikipedia (2015) https://en.wikipedia.org/wiki/Langerhans_cell_histiocytosis Accessed on February, 2 2016</ref><ref name="radio">Langerhans cell histiocytosis. Radiopeadia (2015) http://radiopaedia.org/articles/langerhans-cell-histiocytosis Accessed on February, 3 2016</ref>

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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.[1]
  • 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.

Complications

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.[2][3][1][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. ↑ 1.0 1.1 1.2 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.
  2. ↑ 2.0 2.1 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.
  3. ↑ 3.0 3.1 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
  4. ↑ 4.0 4.1 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. ↑ 5.0 5.1 Langerhans cell histiocytosis. Wikipedia (2015) https://en.wikipedia.org/wiki/Langerhans_cell_histiocytosis Accessed on February, 2 2016
  6. ↑ 6.0 6.1 Langerhans cell histiocytosis. Radiopeadia (2015) http://radiopaedia.org/articles/langerhans-cell-histiocytosis Accessed on February, 3 2016


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