Langerhans cell histiocytosis natural history: Difference between revisions

Jump to navigation Jump to search
VisualWikitext
No edit summary
 
(26 intermediate revisions by 6 users not shown)
Line 1: Line 1:
__NOTOC__
__NOTOC__
{{Langerhans cell histiocytosis}}
{{Langerhans cell histiocytosis}}
{{CMG}} {{AE}} {{HL}}
==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.
==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>
* 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.<ref>{{Cite journal
| author = [[Y. Fukuda]], [[F. Basset]], [[P. Soler]], [[V. J. Ferrans]], [[Y. Masugi]] & [[R. G. Crystal]]
| title = Intraluminal fibrosis and elastic fiber degradation lead to lung remodeling in pulmonary Langerhans cell granulomatosis (histiocytosis X)
| journal = [[The American journal of pathology]]
| volume = 137
| issue = 2
| pages = 415–424
| year = 1990
| month = August
| pmid = 2386203
}}</ref><ref>{{Cite journal
| author = [[P. Soler]], [[M. Kambouchner]], [[D. Valeyre]] & [[A. J. Hance]]
| title = Pulmonary Langerhans' cell granulomatosis (histiocytosis X)
| journal = [[Annual review of medicine]]
| volume = 43
| pages = 105–115
| year = 1992
| month =
| doi = 10.1146/annurev.me.43.020192.000541
| pmid = 1580576
}}</ref><ref>{{Cite journal
| author = [[D. M. Howarth]], [[G. S. Gilchrist]], [[B. P. Mullan]], [[G. A. Wiseman]], [[J. H. Edmonson]] & [[P. J. Schomberg]]
| title = Langerhans cell histiocytosis: diagnosis, natural history, management, and outcome
| journal = [[Cancer]]
| volume = 85
| issue = 10
| pages = 2278–2290
| year = 1999
| month = May
| pmid = 10326709
}}</ref>
==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>
:* [[Pulmonary fibrosis]]
:* [[Pulmonary hypertension]]
:* Pathological [[bone fracture]]s
:* [[Anemia]]
:* Recurrent [[bleeding]]
:* Recurrent [[infection]]s
:* 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.<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 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:
{| style="border: 0px; font-size: 90%; margin: 3px; width: 1000px"
| valign="top" |
|+
! style="background: #4479BA; width: 250px; color: #FFFFFF;" |'''Prognostic Factor'''
! 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;" |
:* 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;" |
:* A more extensive organ involvement is associated with a worse prognosis.
|-


Please help WikiDoc by adding content here.  It's easy!  Click  [[Help:How_to_Edit_a_Page|here]] to learn about editing.
| 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.


==Overview==
|-
 
| 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.


==Prognosis==
|}
Excellent for single foci disease.
<br />With multi-focal disease 60% have a chronic course, 30% achieve remission and mortality is up to 10%<ref>{{cite journal |author=Komp D, El Mahdi A, Starling K, Easley J, Vietti T, Berry D, George S |title=Quality of survival in histiocytosis X: a Southwest Oncology Group study |journal=Med. Pediatr. Oncol. |volume=8 |issue=1 |pages=35-40 |year=1980 |pmid=6969347}}</ref>.


==References==
==References==
Line 18: Line 88:
[[Category:Disease]]
[[Category:Disease]]
[[Category:Autoimmune diseases]]
[[Category:Autoimmune diseases]]
[[Category:Hematology]]
[[Category:Up-To-Date]]
[[Category:Oncology]]
[[Category:Oncology]]
[[Category:Hematology]]
[[Category:Medicine]]
[[Category:Dermatology]]
[[Category:Surgery]]

Latest revision as of 19:12, 25 March 2019

Langerhans cell histiocytosis Microchapters

Home

Patient Information

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Langerhans cell histiocytosis from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

History and Symptoms

Physical Examination

Laboratory Findings

Chest X Ray

CT

MRI

Echocardiography or Ultrasound

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

Langerhans cell histiocytosis natural history On the Web

Most recent articles

Most cited articles

Review articles

CME Programs

Powerpoint slides

Images

American Roentgen Ray Society Images of Langerhans cell histiocytosis natural history

All Images
X-rays
Echo & Ultrasound
CT Images
MRI

Ongoing Trials at Clinical Trials.gov

US National Guidelines Clearinghouse

NICE Guidance

FDA on Langerhans cell histiocytosis natural history

on Langerhans cell histiocytosis natural history

Langerhans cell histiocytosis natural history in the news

Blogs on Langerhans cell histiocytosis natural history

Directions to Hospitals Treating Langerhans cell histiocytosis

Risk calculators and risk factors for Langerhans cell histiocytosis natural history

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. Pulmonary LCH typicaly presents as bronchiolitis that may progress to intraluminal fibrosis and elastic fiber degradation and lung remodeling.[2][3][4]

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.[5][6][1][7][8][9]
  • 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. ↑ Y. Fukuda, F. Basset, P. Soler, V. J. Ferrans, Y. Masugi & R. G. Crystal (1990). "Intraluminal fibrosis and elastic fiber degradation lead to lung remodeling in pulmonary Langerhans cell granulomatosis (histiocytosis X)". The American journal of pathology. 137 (2): 415–424. PMID 2386203. Unknown parameter |month= ignored (help)
  3. ↑ P. Soler, M. Kambouchner, D. Valeyre & A. J. Hance (1992). "Pulmonary Langerhans' cell granulomatosis (histiocytosis X)". Annual review of medicine. 43: 105–115. doi:10.1146/annurev.me.43.020192.000541. PMID 1580576.
  4. ↑ D. M. Howarth, G. S. Gilchrist, B. P. Mullan, G. A. Wiseman, J. H. Edmonson & P. J. Schomberg (1999). "Langerhans cell histiocytosis: diagnosis, natural history, management, and outcome". Cancer. 85 (10): 2278–2290. PMID 10326709. Unknown parameter |month= ignored (help)
  5. ↑ 5.0 5.1 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.
  6. ↑ 6.0 6.1 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
  7. ↑ 7.0 7.1 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.
  8. ↑ 8.0 8.1 Langerhans cell histiocytosis. Wikipedia (2015) https://en.wikipedia.org/wiki/Langerhans_cell_histiocytosis Accessed on February, 2 2016
  9. ↑ 9.0 9.1 Langerhans cell histiocytosis. Radiopeadia (2015) http://radiopaedia.org/articles/langerhans-cell-histiocytosis Accessed on February, 3 2016


Template:WikiDoc Sources

Retrieved from "https://www.wikidoc.org/index.php?title=Langerhans_cell_histiocytosis_natural_history&oldid=1559100"