Langerhans cell histiocytosis natural history: Difference between revisions
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* 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: | |||
{| style="border: 0px; font-size: 90%; margin: 3px; width: 1000px" | |||
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! style="background: #4479BA; width: 250px; color: #FFFFFF;"|'''Prognostic Factor''' | |||
! style="background: #4479BA; width: 600px; color: #FFFFFF;"|'''Description''' | |||
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| 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. | |||
|- | |||
| 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. | |||
|- | |||
| 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. | |||
|} | |||
==References== | ==References== | ||
Revision as of 16:32, 4 February 2016
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Langerhans cell histiocytosis Microchapters |
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Differentiating Langerhans cell histiocytosis from other Diseases |
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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
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 |
|