Langerhans cell histiocytosis natural history: Difference between revisions

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==Overview==
==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 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.<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>
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>   
==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>
:* [[Pulmonary fibrosis]]
:* [[Pulmonary fibrosis]]
:* [[Pulmonary hypertension]]  
:* [[Pulmonary hypertension]]  
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:* Central [[diabetes insipidus]]
:* Central [[diabetes insipidus]]
==Prognosis==
==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.<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>
* Depending on the extent of the [[tumor]] at the time of [[diagnosis]], the [[prognosis]] may vary. However, the [[prognosis]] is generally regarded as excellent.<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>
* The  5-year survival rate of patients with unifocal Langerhans cell histiocytosis is approximately 99%.
* 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 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  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:
* The table below lists [[prognostic]] factors for Langerhans cell histiocytosis patients:
{| style="border: 0px; font-size: 90%; margin: 3px; width: 1000px"
{| style="border: 0px; font-size: 90%; margin: 3px; width: 1000px"
|valign=top|
| valign="top" |
|+
|+
! style="background: #4479BA; width: 250px; color: #FFFFFF;"|'''Prognostic Factor'''
! style="background: #4479BA; width: 250px; color: #FFFFFF;" |'''Prognostic Factor'''


! style="background: #4479BA; width: 600px; color: #FFFFFF;"|'''Description'''
! style="background: #4479BA; width: 600px; color: #FFFFFF;" |'''Description'''


|-
|-


| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align=center | '''Age'''|| style="padding: 5px 5px; background: #F5F5F5;" |
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" | '''Age'''|| style="padding: 5px 5px; background: #F5F5F5;" |
:* A younger age at the time of diagnosis is associated with a worse prognosis.
:* A younger age at the time of diagnosis is associated with a worse prognosis.


|-
|-


| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align=center | '''Organ involvement'''|| style="padding: 5px 5px; background: #F5F5F5;"|
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" | '''Organ involvement'''|| style="padding: 5px 5px; background: #F5F5F5;" |
:* A more extensive organ involvement is associated with a worse prognosis.
:* A more extensive organ involvement is associated with a worse prognosis.
|-
|-


| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align=center | '''Response to treatment'''|| style="padding: 5px 5px; background: #F5F5F5;" |
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" | '''Response to treatment'''|| style="padding: 5px 5px; background: #F5F5F5;" |
:*A  modest response rate after 6 weeks of therapy is associated with a worse prognosis.
:*A  modest response rate after 6 weeks of therapy is associated with a worse prognosis.


|-
|-


| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align=center | '''Cellular markers'''|| style="padding: 5px 5px; background: #F5F5F5;" |
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" | '''Cellular markers'''|| style="padding: 5px 5px; background: #F5F5F5;" |
:* The expression of [[metalloproteinase]] and gelosin on a cellular level is associated with a worse prognosis.
:* The expression of [[metalloproteinase]] and gelosin on a cellular level is associated with a worse prognosis.


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Revision as of 18:58, 25 March 2019

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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]

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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