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="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> | 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="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> | ||
==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. | * 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. | ||
Revision as of 16:36, 4 February 2016
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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]
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%.
- The table below lists prognostic factors for Langerhans cell histiocytosis patients:
| Prognostic Factor | Description |
|---|---|
| Age |
|
| Organ involvement |
|
| Response to treatment |
|
| Cellular markers |
|
References
- ↑ 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
- ↑ 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.
- ↑ 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.