Autoimmune lymphoproliferative syndrome: Difference between revisions
| Line 25: | Line 25: | ||
==[[Autoimmune lymphoproliferative syndrome historical perspective|Historical Perspective]]== | ==[[Autoimmune lymphoproliferative syndrome historical perspective|Historical Perspective]]== | ||
==Discovery== | ==Discovery== | ||
<br /> | <br /> | ||
== Overview == | ==Overview== | ||
The exact pathogenesis of [disease name] is not fully understood. | The exact pathogenesis of [disease name] is not fully understood. | ||
| Line 62: | Line 56: | ||
The pathophysiology of [disease/malignancy] depends on the histological subtype. | The pathophysiology of [disease/malignancy] depends on the histological subtype. | ||
== Pathophysiology == | ==Pathophysiology== | ||
=== Physiology === | ===Physiology=== | ||
The normal physiology of [name of process] can be understood as follows: | The normal physiology of [name of process] can be understood as follows: | ||
=== Pathogenesis === | ===Pathogenesis=== | ||
* The exact pathogenesis of [disease name] is not completely understood. | *The exact pathogenesis of [disease name] is not completely understood. | ||
OR | OR | ||
* It is understood that [disease name] is the result of / is mediated by / is produced by / is caused by either [hypothesis 1], [hypothesis 2], or [hypothesis 3]. | *It is understood that [disease name] is the result of / is mediated by / is produced by / is caused by either [hypothesis 1], [hypothesis 2], or [hypothesis 3]. | ||
* [Pathogen name] is usually transmitted via the [transmission route] route to the human host. | *[Pathogen name] is usually transmitted via the [transmission route] route to the human host. | ||
* Following transmission/ingestion, the [pathogen] uses the [entry site] to invade the [cell name] cell. | *Following transmission/ingestion, the [pathogen] uses the [entry site] to invade the [cell name] cell. | ||
* [Disease or malignancy name] arises from [cell name]s, which are [cell type] cells that are normally involved in [function of cells]. | *[Disease or malignancy name] arises from [cell name]s, which are [cell type] cells that are normally involved in [function of cells]. | ||
* The progression to [disease name] usually involves the [molecular pathway]. | *The progression to [disease name] usually involves the [molecular pathway]. | ||
* The pathophysiology of [disease/malignancy] depends on the histological subtype. | *The pathophysiology of [disease/malignancy] depends on the histological subtype. | ||
== Genetics == | ==Genetics== | ||
[Disease name] is transmitted in [mode of genetic transmission] pattern. | [Disease name] is transmitted in [mode of genetic transmission] pattern. | ||
| Line 87: | Line 81: | ||
Genes involved in the pathogenesis of [disease name] include: | Genes involved in the pathogenesis of [disease name] include: | ||
* [Gene1] | *[Gene1] | ||
* [Gene2] | *[Gene2] | ||
* [Gene3] | *[Gene3] | ||
OR | OR | ||
| Line 95: | Line 89: | ||
The development of [disease name] is the result of multiple genetic mutations such as: | The development of [disease name] is the result of multiple genetic mutations such as: | ||
* [Mutation 1] | *[Mutation 1] | ||
* [Mutation 2] | *[Mutation 2] | ||
* [Mutation 3] | *[Mutation 3] | ||
== Associated Conditions == | ==Associated Conditions== | ||
Conditions associated with [disease name] include: | Conditions associated with [disease name] include: | ||
* [Condition 1] | *[Condition 1] | ||
* [Condition 2] | *[Condition 2] | ||
* [Condition 3] | *[Condition 3] | ||
== Gross Pathology == | ==Gross Pathology== | ||
On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name]. | On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name]. | ||
== Microscopic Pathology == | ==Microscopic Pathology== | ||
On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of [disease name]. | On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of [disease name]. | ||
== References == | ==References== | ||
==[[Autoimmune lymphoproliferative syndrome classification|Classification]]== | ==[[Autoimmune lymphoproliferative syndrome classification|Classification]]== | ||
<br /> | <br /> | ||
== Overview == | ==Overview== | ||
There is no established system for the classification of [disease name]. | There is no established system for the classification of [disease name]. | ||
| Line 144: | Line 138: | ||
There is no established system for the staging of [malignancy name]. | There is no established system for the staging of [malignancy name]. | ||
== Classification == | ==Classification== | ||
There is no established system for the classification of [disease name]. | There is no established system for the classification of [disease name]. | ||
| Line 151: | Line 145: | ||
[Disease name] may be classified according to [classification method] into [number] subtypes/groups: | [Disease name] may be classified according to [classification method] into [number] subtypes/groups: | ||
* [Group1] | *[Group1] | ||
* [Group2] | *[Group2] | ||
* [Group3] | *[Group3] | ||
* [Group4] | *[Group4] | ||
OR | OR | ||
| Line 160: | Line 154: | ||
[Disease name] may be classified into [large number > 6] subtypes based on: | [Disease name] may be classified into [large number > 6] subtypes based on: | ||
* [Classification method 1] | *[Classification method 1] | ||
* [Classification method 2] | *[Classification method 2] | ||
* [Classification method 3] | *[Classification method 3] | ||
[Disease name] may be classified into several subtypes based on: | [Disease name] may be classified into several subtypes based on: | ||
* [Classification method 1] | *[Classification method 1] | ||
* [Classification method 2] | *[Classification method 2] | ||
* [Classification method 3] | *[Classification method 3] | ||
OR | OR | ||
| Line 188: | Line 182: | ||
There is no established system for the staging of [malignancy name]. | There is no established system for the staging of [malignancy name]. | ||
== References == | ==References== | ||
==[[Autoimmune lymphoproliferative syndrome pathophysiology|Pathophysiology]]== | ==[[Autoimmune lymphoproliferative syndrome pathophysiology|Pathophysiology]]== | ||
<br /> | <br /> | ||
== Overview == | ==Overview== | ||
The exact pathogenesis of [disease name] is not fully understood. | The exact pathogenesis of [disease name] is not fully understood. | ||
| Line 220: | Line 214: | ||
The pathophysiology of [disease/malignancy] depends on the histological subtype. | The pathophysiology of [disease/malignancy] depends on the histological subtype. | ||
== Pathophysiology == | ==Pathophysiology== | ||
=== Physiology === | ===Physiology=== | ||
The normal physiology of [name of process] can be understood as follows: | The normal physiology of [name of process] can be understood as follows: | ||
=== Pathogenesis === | ===Pathogenesis=== | ||
* The exact pathogenesis of [disease name] is not completely understood. | *The exact pathogenesis of [disease name] is not completely understood. | ||
OR | OR | ||
* It is understood that [disease name] is the result of / is mediated by / is produced by / is caused by either [hypothesis 1], [hypothesis 2], or [hypothesis 3]. | *It is understood that [disease name] is the result of / is mediated by / is produced by / is caused by either [hypothesis 1], [hypothesis 2], or [hypothesis 3]. | ||
* [Pathogen name] is usually transmitted via the [transmission route] route to the human host. | *[Pathogen name] is usually transmitted via the [transmission route] route to the human host. | ||
* Following transmission/ingestion, the [pathogen] uses the [entry site] to invade the [cell name] cell. | *Following transmission/ingestion, the [pathogen] uses the [entry site] to invade the [cell name] cell. | ||
* [Disease or malignancy name] arises from [cell name]s, which are [cell type] cells that are normally involved in [function of cells]. | *[Disease or malignancy name] arises from [cell name]s, which are [cell type] cells that are normally involved in [function of cells]. | ||
* The progression to [disease name] usually involves the [molecular pathway]. | *The progression to [disease name] usually involves the [molecular pathway]. | ||
* The pathophysiology of [disease/malignancy] depends on the histological subtype. | *The pathophysiology of [disease/malignancy] depends on the histological subtype. | ||
== Genetics == | ==Genetics== | ||
[Disease name] is transmitted in [mode of genetic transmission] pattern. | [Disease name] is transmitted in [mode of genetic transmission] pattern. | ||
| Line 245: | Line 239: | ||
Genes involved in the pathogenesis of [disease name] include: | Genes involved in the pathogenesis of [disease name] include: | ||
* [Gene1] | *[Gene1] | ||
* [Gene2] | *[Gene2] | ||
* [Gene3] | *[Gene3] | ||
OR | OR | ||
| Line 253: | Line 247: | ||
The development of [disease name] is the result of multiple genetic mutations such as: | The development of [disease name] is the result of multiple genetic mutations such as: | ||
* [Mutation 1] | *[Mutation 1] | ||
* [Mutation 2] | *[Mutation 2] | ||
* [Mutation 3] | *[Mutation 3] | ||
== Associated Conditions == | ==Associated Conditions== | ||
Conditions associated with [disease name] include: | Conditions associated with [disease name] include: | ||
* [Condition 1] | *[Condition 1] | ||
* [Condition 2] | *[Condition 2] | ||
* [Condition 3] | *[Condition 3] | ||
== Gross Pathology == | ==Gross Pathology== | ||
On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name]. | On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name]. | ||
== Microscopic Pathology == | ==Microscopic Pathology== | ||
On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of [disease name]. | On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of [disease name]. | ||
== References == | ==References== | ||
==[[Autoimmune lymphoproliferative syndrome causes|Causes]]== | ==[[Autoimmune lymphoproliferative syndrome causes|Causes]]== | ||
<br /> | <br /> | ||
== Overview[edit | edit source] == | ==Overview[edit | edit source]== | ||
Disease name] may be caused by [cause1], [cause2], or [cause3]. | Disease name] may be caused by [cause1], [cause2], or [cause3]. | ||
| Line 290: | Line 284: | ||
The cause of [disease name] has not been identified. To review risk factors for the development of [disease name], click here. | The cause of [disease name] has not been identified. To review risk factors for the development of [disease name], click here. | ||
== Causes[edit | edit source] == | ==Causes[edit | edit source]== | ||
* ymptom/manifestation] include [cause1], [cause2], and [cause3]. | *ymptom/manifestation] include [cause1], [cause2], and [cause3]. | ||
* [Cause] is a life-threatening cause of [disease]. | *[Cause] is a life-threatening cause of [disease]. | ||
=== Common Causes[edit | edit source] === | ===Common Causes[edit | edit source]=== | ||
Common causes of [disease name] may include: | Common causes of [disease name] may include: | ||
* [Cause1] | *[Cause1] | ||
* [Cause2] | *[Cause2] | ||
* [Cause3] | *[Cause3] | ||
OR | OR | ||
* [Disease name] is caused by an infection with [pathogen name]. | *[Disease name] is caused by an infection with [pathogen name]. | ||
* [Pathogen name] is caused by [pathogen name]. | *[Pathogen name] is caused by [pathogen name]. | ||
=== Less Common Causes[edit | edit source] === | ===Less Common Causes[edit | edit source]=== | ||
Less common causes of [disease name] include: | Less common causes of [disease name] include: | ||
* [Cause1] | *[Cause1] | ||
* [Cause2] | *[Cause2] | ||
* [CauseCauses by OrganList the causes of the disease in alphabetical order: | *[CauseCauses by OrganList the causes of the disease in alphabetical order: | ||
* Cause 1 | *Cause 1 | ||
* Cause 2 | *Cause 2 | ||
* Cause 3 | *Cause 3 | ||
* Cause 4 | *Cause 4 | ||
* Cause 5 | *Cause 5 | ||
* Cause 6 | *Cause 6 | ||
* Cause 7 | *Cause 7 | ||
* Cause 8 | *Cause 8 | ||
* Cause 9 | *Cause 9 | ||
* Cause 10 | *Cause 10 | ||
== References == | ==References== | ||
==[[Autoimmune lymphoproliferative syndrome differential diagnosis|Differentiating Autoimmune lymphoproliferative syndrome from other Diseases]]== | ==[[Autoimmune lymphoproliferative syndrome differential diagnosis|Differentiating Autoimmune lymphoproliferative syndrome from other Diseases]]== | ||
<br /> | <br /> | ||
== Overview == | ==Overview== | ||
[Disease name] must be differentiated from other diseases that cause [clinical feature 1], [clinical feature 2], and [clinical feature 3], such as [differential dx1], [differential dx2], and [differential dx3]. | [Disease name] must be differentiated from other diseases that cause [clinical feature 1], [clinical feature 2], and [clinical feature 3], such as [differential dx1], [differential dx2], and [differential dx3]. | ||
| Line 336: | Line 330: | ||
[Disease name] must be differentiated from [[differential dx1], [differential dx2], and [differential dx3]. | [Disease name] must be differentiated from [[differential dx1], [differential dx2], and [differential dx3]. | ||
== Differentiating [Disease name] from other Diseases == | ==Differentiating [Disease name] from other Diseases== | ||
[Disease name] must be differentiated from other diseases that cause [clinical feature 1], [clinical feature 2], and [clinical feature 3], such as [differential dx1], [differential dx2], and [differential dx3]. | [Disease name] must be differentiated from other diseases that cause [clinical feature 1], [clinical feature 2], and [clinical feature 3], such as [differential dx1], [differential dx2], and [differential dx3]. | ||
| Line 347: | Line 341: | ||
As [disease name] manifests in a variety of clinical forms, differentiation must be established in accordance with the particular subtype. [Subtype name 1] must be differentiated from other diseases that cause [clinical feature 1], such as [differential dx1] and [differential dx2]. In contrast, [subtype name 2] must be differentiated from other diseases that cause [clinical feature 2], such as [differential dx3] and [differential dx4]. | As [disease name] manifests in a variety of clinical forms, differentiation must be established in accordance with the particular subtype. [Subtype name 1] must be differentiated from other diseases that cause [clinical feature 1], such as [differential dx1] and [differential dx2]. In contrast, [subtype name 2] must be differentiated from other diseases that cause [clinical feature 2], such as [differential dx3] and [differential dx4]. | ||
=== Differentiating [disease name] from other diseases on the basis of [symptom 1], [symptom 2], and [symptom 3] === | ===Differentiating [disease name] from other diseases on the basis of [symptom 1], [symptom 2], and [symptom 3]=== | ||
On the basis [symptom 1], [symptom 2], and [symptom 3], [disease name] must be differentiated from [disease 1], [disease 2], [disease 3], [disease 4], [disease 5], and [disease 6]. | On the basis [symptom 1], [symptom 2], and [symptom 3], [disease name] must be differentiated from [disease 1], [disease 2], [disease 3], [disease 4], [disease 5], and [disease 6]. | ||
{| class="wikitable" | {| class="wikitable" | ||
| Line 496: | Line 490: | ||
|} | |} | ||
== References == | ==References== | ||
==[[Autoimmune lymphoproliferative syndrome epidemiology and demographics|Epidemiology and Demographics]]== | ==[[Autoimmune lymphoproliferative syndrome epidemiology and demographics|Epidemiology and Demographics]]== | ||
== Overview == | ==Overview== | ||
== Epidemiology and Demographics == | ==Epidemiology and Demographics== | ||
=== Incidence === | ===Incidence=== | ||
* The incidence/prevalence of [disease name] is approximately [number range] per 100,000 individuals worldwide. | *The incidence/prevalence of [disease name] is approximately [number range] per 100,000 individuals worldwide. | ||
* In [year], the incidence/prevalence of [disease name] was estimated to be [number range] cases per 100,000 individuals worldwide. | *In [year], the incidence/prevalence of [disease name] was estimated to be [number range] cases per 100,000 individuals worldwide. | ||
=== Prevalence === | ===Prevalence=== | ||
* The incidence/prevalence of [disease name] is approximately [number range] per 100,000 individuals worldwide. | *The incidence/prevalence of [disease name] is approximately [number range] per 100,000 individuals worldwide. | ||
* In [year], the incidence/prevalence of [disease name] was estimated to be [number range] cases per 100,000 individuals worldwide. | *In [year], the incidence/prevalence of [disease name] was estimated to be [number range] cases per 100,000 individuals worldwide. | ||
* The prevalence of [disease/malignancy] is estimated to be [number] cases annually. | *The prevalence of [disease/malignancy] is estimated to be [number] cases annually. | ||
=== Case-fatality rate/Mortality rate === | ===Case-fatality rate/Mortality rate=== | ||
* In [year], the incidence of [disease name] is approximately [number range] per 100,000 individuals with a case-fatality rate/mortality rate of [number range]%. | *In [year], the incidence of [disease name] is approximately [number range] per 100,000 individuals with a case-fatality rate/mortality rate of [number range]%. | ||
* The case-fatality rate/mortality rate of [disease name] is approximately [number range]. | *The case-fatality rate/mortality rate of [disease name] is approximately [number range]. | ||
=== Age === | ===Age=== | ||
* Patients of all age groups may develop [disease name]. | *Patients of all age groups may develop [disease name]. | ||
* The incidence of [disease name] increases with age; the median age at diagnosis is [#] years. | *The incidence of [disease name] increases with age; the median age at diagnosis is [#] years. | ||
* [Disease name] commonly affects individuals younger than/older than [number of years] years of age. | *[Disease name] commonly affects individuals younger than/older than [number of years] years of age. | ||
* [Chronic disease name] is usually first diagnosed among [age group]. | *[Chronic disease name] is usually first diagnosed among [age group]. | ||
* [Acute disease name] commonly affects [age group]. | *[Acute disease name] commonly affects [age group]. | ||
=== Race === | ===Race=== | ||
* There is no racial predilection to [disease name]. | *There is no racial predilection to [disease name]. | ||
* [Disease name] usually affects individuals of the [race 1] race. [Race 2] individuals are less likely to develop [disease name]. | *[Disease name] usually affects individuals of the [race 1] race. [Race 2] individuals are less likely to develop [disease name]. | ||
=== Gender === | ===Gender=== | ||
* [Disease name] affects men and women equally. | *[Disease name] affects men and women equally. | ||
* [Gender 1] are more commonly affected by [disease name] than [gender 2]. The [gender 1] to [gender 2] ratio is approximately [number > 1] to 1. | *[Gender 1] are more commonly affected by [disease name] than [gender 2]. The [gender 1] to [gender 2] ratio is approximately [number > 1] to 1. | ||
=== Region === | ===Region=== | ||
* The majority of [disease name] cases are reported in [geographical region]. | *The majority of [disease name] cases are reported in [geographical region]. | ||
* [Disease name] is a common/rare disease that tends to affect [patient population 1] and [patient population 2]. | *[Disease name] is a common/rare disease that tends to affect [patient population 1] and [patient population 2]. | ||
=== Developed Countries === | ===Developed Countries=== | ||
=== Developing Countries === | ===Developing Countries=== | ||
== References == | ==References== | ||
==[[Autoimmune lymphoproliferative syndrome risk factors|Risk Factors]]== | ==[[Autoimmune lymphoproliferative syndrome risk factors|Risk Factors]]== | ||
== Overview == | ==Overview== | ||
There are no established risk factors for [disease name]. | There are no established risk factors for [disease name]. | ||
| Line 567: | Line 561: | ||
Common risk factors in the development of [disease name] may be occupational, environmental, genetic, and viral. | Common risk factors in the development of [disease name] may be occupational, environmental, genetic, and viral. | ||
== Risk Factors == | ==Risk Factors== | ||
There are no established risk factors for [disease name]. | There are no established risk factors for [disease name]. | ||
| Line 578: | Line 572: | ||
Common risk factors in the development of [disease name] include [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4]. | Common risk factors in the development of [disease name] include [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4]. | ||
=== Common Risk Factors === | ===Common Risk Factors=== | ||
* Common risk factors in the development of [disease name] may be occupational, environmental, genetic, and viral. | *Common risk factors in the development of [disease name] may be occupational, environmental, genetic, and viral. | ||
* Common risk factors in the development of [disease name] include: | *Common risk factors in the development of [disease name] include: | ||
** [Risk factor 1] | **[Risk factor 1] | ||
** [Risk factor 2] | **[Risk factor 2] | ||
** [Risk factor 3] | **[Risk factor 3] | ||
=== Less Common Risk Factors === | ===Less Common Risk Factors=== | ||
* Less common risk factors in the development of [disease name] include: | *Less common risk factors in the development of [disease name] include: | ||
** [Risk factor 1] | **[Risk factor 1] | ||
** [Risk factor 2] | **[Risk factor 2] | ||
** [Risk factor 3] | **[Risk factor 3] | ||
== References == | ==References== | ||
==[[Autoimmune lymphoproliferative syndrome screening|Screening]]== | ==[[Autoimmune lymphoproliferative syndrome screening|Screening]]== | ||
== Overview == | ==Overview== | ||
There is insufficient evidence to recommend routine screening for [disease/malignancy]. | There is insufficient evidence to recommend routine screening for [disease/malignancy]. | ||
| Line 608: | Line 602: | ||
According to the [guideline name], screening for [disease name] by [test 1] is recommended every [duration] among patients with [condition 1], [condition 2], and [condition 3]. | According to the [guideline name], screening for [disease name] by [test 1] is recommended every [duration] among patients with [condition 1], [condition 2], and [condition 3]. | ||
== Screening == | ==Screening== | ||
There is insufficient evidence to recommend routine screening for [disease/malignancy]. | There is insufficient evidence to recommend routine screening for [disease/malignancy]. | ||
| Line 619: | Line 613: | ||
According to the [guideline name], screening for [disease name] by [test 1] is recommended every [duration] among patients with: | According to the [guideline name], screening for [disease name] by [test 1] is recommended every [duration] among patients with: | ||
* [Condition 1] | *[Condition 1] | ||
* [Condition 2] | *[Condition 2] | ||
* [Condition 3] | *[Condition 3] | ||
== References == | ==References== | ||
==[[Autoimmune lymphoproliferative syndrome natural history, complications and prognosis|Natural History, Complications and Prognosis]]== | ==[[Autoimmune lymphoproliferative syndrome natural history, complications and prognosis|Natural History, Complications and Prognosis]]== | ||
== Overview == | ==Overview== | ||
If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3]. | If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3]. | ||
| Line 638: | Line 632: | ||
Prognosis is generally excellent/good/poor, and the 1/5/10-year mortality/survival rate of patients with [disease name] is approximately [#]%. | Prognosis is generally excellent/good/poor, and the 1/5/10-year mortality/survival rate of patients with [disease name] is approximately [#]%. | ||
== Natural History, Complications, and Prognosis == | ==Natural History, Complications, and Prognosis== | ||
=== Natural History === | ===Natural History=== | ||
* The symptoms of (disease name) usually develop in the first/ second/ third decade of life, and start with symptoms such as ___. | *The symptoms of (disease name) usually develop in the first/ second/ third decade of life, and start with symptoms such as ___. | ||
* The symptoms of (disease name) typically develop ___ years after exposure to ___. | *The symptoms of (disease name) typically develop ___ years after exposure to ___. | ||
* If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3]. | *If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3]. | ||
=== Complications === | ===Complications=== | ||
* Common complications of [disease name] include: | *Common complications of [disease name] include: | ||
** [Complication 1] | **[Complication 1] | ||
** [Complication 2] | **[Complication 2] | ||
** [Complication 3] | **[Complication 3] | ||
=== Prognosis === | ===Prognosis=== | ||
* Prognosis is generally excellent/good/poor, and the 1/5/10-year mortality/survival rate of patients with [disease name] is approximately [--]%. | *Prognosis is generally excellent/good/poor, and the 1/5/10-year mortality/survival rate of patients with [disease name] is approximately [--]%. | ||
* Depending on the extent of the [tumor/disease progression] at the time of diagnosis, the prognosis may vary. However, the prognosis is generally regarded as poor/good/excellent. | *Depending on the extent of the [tumor/disease progression] at the time of diagnosis, the prognosis may vary. However, the prognosis is generally regarded as poor/good/excellent. | ||
* The presence of [characteristic of disease] is associated with a particularly [good/poor] prognosis among patients with [disease/malignancy]. | *The presence of [characteristic of disease] is associated with a particularly [good/poor] prognosis among patients with [disease/malignancy]. | ||
* [Subtype of disease/malignancy] is associated with the most favorable prognosis. | *[Subtype of disease/malignancy] is associated with the most favorable prognosis. | ||
* The prognosis varies with the [characteristic] of tumor; [subtype of disease/malignancy] have the most favorable prognosis. | *The prognosis varies with the [characteristic] of tumor; [subtype of disease/malignancy] have the most favorable prognosis. | ||
== References == | ==References== | ||
==Diagnosis== | ==Diagnosis== | ||
Revision as of 01:56, 22 June 2021
| Autoimmune lymphoproliferative syndrome | |
| OMIM | 601859 603909 |
|---|---|
| DiseasesDB | 33425 Template:DiseasesDB2 |
|
Autoimmune lymphoproliferative syndrome Microchapters |
|
Differentiating Autoimmune lymphoproliferative syndrome from other Diseases |
|---|
|
Diagnosis |
|
Treatment |
|
Case Studies |
|
Autoimmune lymphoproliferative syndrome On the Web |
|
American Roentgen Ray Society Images of Autoimmune lymphoproliferative syndrome |
|
Directions to Hospitals Treating Autoimmune lymphoproliferative syndrome |
|
Risk calculators and risk factors for Autoimmune lymphoproliferative syndrome |
Editor-In-Chief: David Teachey, MD [1]
Synonyms and keywords: Canale-Smith syndrome; ALPS
Overview
Historical Perspective
Discovery
Overview
The exact pathogenesis of [disease name] is not fully understood.
OR
It is thought that [disease name] is the result of / is mediated by / is produced by / is caused by either [hypothesis 1], [hypothesis 2], or [hypothesis 3].
OR
[Pathogen name] is usually transmitted via the [transmission route] route to the human host.
OR
Following transmission/ingestion, the [pathogen] uses the [entry site] to invade the [cell name] cell.
OR
[Disease or malignancy name] arises from [cell name]s, which are [cell type] cells that are normally involved in [function of cells].
OR
The progression to [disease name] usually involves the [molecular pathway].
OR
The pathophysiology of [disease/malignancy] depends on the histological subtype.
Pathophysiology
Physiology
The normal physiology of [name of process] can be understood as follows:
Pathogenesis
- The exact pathogenesis of [disease name] is not completely understood.
OR
- It is understood that [disease name] is the result of / is mediated by / is produced by / is caused by either [hypothesis 1], [hypothesis 2], or [hypothesis 3].
- [Pathogen name] is usually transmitted via the [transmission route] route to the human host.
- Following transmission/ingestion, the [pathogen] uses the [entry site] to invade the [cell name] cell.
- [Disease or malignancy name] arises from [cell name]s, which are [cell type] cells that are normally involved in [function of cells].
- The progression to [disease name] usually involves the [molecular pathway].
- The pathophysiology of [disease/malignancy] depends on the histological subtype.
Genetics
[Disease name] is transmitted in [mode of genetic transmission] pattern.
OR
Genes involved in the pathogenesis of [disease name] include:
- [Gene1]
- [Gene2]
- [Gene3]
OR
The development of [disease name] is the result of multiple genetic mutations such as:
- [Mutation 1]
- [Mutation 2]
- [Mutation 3]
Associated Conditions
Conditions associated with [disease name] include:
- [Condition 1]
- [Condition 2]
- [Condition 3]
Gross Pathology
On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
Microscopic Pathology
On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
References
Classification
Overview
There is no established system for the classification of [disease name].
OR
[Disease name] may be classified according to [classification method] into [number] subtypes/groups: [group1], [group2], [group3], and [group4].
OR
[Disease name] may be classified into [large number > 6] subtypes based on [classification method 1], [classification method 2], and [classification method 3]. [Disease name] may be classified into several subtypes based on [classification method 1], [classification method 2], and [classification method 3].
OR
Based on the duration of symptoms, [disease name] may be classified as either acute or chronic.
OR
If the staging system involves specific and characteristic findings and features: According to the [staging system + reference], there are [number] stages of [malignancy name] based on the [finding1], [finding2], and [finding3]. Each stage is assigned a [letter/number1] and a [letter/number2] that designate the [feature1] and [feature2].
OR
The staging of [malignancy name] is based on the [staging system].
OR
There is no established system for the staging of [malignancy name].
Classification
There is no established system for the classification of [disease name].
OR
[Disease name] may be classified according to [classification method] into [number] subtypes/groups:
- [Group1]
- [Group2]
- [Group3]
- [Group4]
OR
[Disease name] may be classified into [large number > 6] subtypes based on:
- [Classification method 1]
- [Classification method 2]
- [Classification method 3]
[Disease name] may be classified into several subtypes based on:
- [Classification method 1]
- [Classification method 2]
- [Classification method 3]
OR
Based on the duration of symptoms, [disease name] may be classified as either acute or chronic.
OR
If the staging system involves specific and characteristic findings and features:
According to the [staging system + reference], there are [number] stages of [malignancy name] based on the [finding1], [finding2], and [finding3]. Each stage is assigned a [letter/number1] and a [letter/number2] that designate the [feature1] and [feature2].
OR
The staging of [malignancy name] is based on the [staging system].
OR
There is no established system for the staging of [malignancy name].
References
Pathophysiology
Overview
The exact pathogenesis of [disease name] is not fully understood.
OR
It is thought that [disease name] is the result of / is mediated by / is produced by / is caused by either [hypothesis 1], [hypothesis 2], or [hypothesis 3].
OR
[Pathogen name] is usually transmitted via the [transmission route] route to the human host.
OR
Following transmission/ingestion, the [pathogen] uses the [entry site] to invade the [cell name] cell.
OR
[Disease or malignancy name] arises from [cell name]s, which are [cell type] cells that are normally involved in [function of cells].
OR
The progression to [disease name] usually involves the [molecular pathway].
OR
The pathophysiology of [disease/malignancy] depends on the histological subtype.
Pathophysiology
Physiology
The normal physiology of [name of process] can be understood as follows:
Pathogenesis
- The exact pathogenesis of [disease name] is not completely understood.
OR
- It is understood that [disease name] is the result of / is mediated by / is produced by / is caused by either [hypothesis 1], [hypothesis 2], or [hypothesis 3].
- [Pathogen name] is usually transmitted via the [transmission route] route to the human host.
- Following transmission/ingestion, the [pathogen] uses the [entry site] to invade the [cell name] cell.
- [Disease or malignancy name] arises from [cell name]s, which are [cell type] cells that are normally involved in [function of cells].
- The progression to [disease name] usually involves the [molecular pathway].
- The pathophysiology of [disease/malignancy] depends on the histological subtype.
Genetics
[Disease name] is transmitted in [mode of genetic transmission] pattern.
OR
Genes involved in the pathogenesis of [disease name] include:
- [Gene1]
- [Gene2]
- [Gene3]
OR
The development of [disease name] is the result of multiple genetic mutations such as:
- [Mutation 1]
- [Mutation 2]
- [Mutation 3]
Associated Conditions
Conditions associated with [disease name] include:
- [Condition 1]
- [Condition 2]
- [Condition 3]
Gross Pathology
On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
Microscopic Pathology
On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
References
Causes
Overview[edit | edit source]
Disease name] may be caused by [cause1], [cause2], or [cause3].
OR
Common causes of [disease] include [cause1], [cause2], and [cause3].
OR
The most common cause of [disease name] is [cause 1]. Less common causes of [disease name] include [cause 2], [cause 3], and [cause 4].
OR
The cause of [disease name] has not been identified. To review risk factors for the development of [disease name], click here.
Causes[edit | edit source]
- ymptom/manifestation] include [cause1], [cause2], and [cause3].
- [Cause] is a life-threatening cause of [disease].
Common Causes[edit | edit source]
Common causes of [disease name] may include:
- [Cause1]
- [Cause2]
- [Cause3]
OR
- [Disease name] is caused by an infection with [pathogen name].
- [Pathogen name] is caused by [pathogen name].
Less Common Causes[edit | edit source]
Less common causes of [disease name] include:
- [Cause1]
- [Cause2]
- [CauseCauses by OrganList the causes of the disease in alphabetical order:
- Cause 1
- Cause 2
- Cause 3
- Cause 4
- Cause 5
- Cause 6
- Cause 7
- Cause 8
- Cause 9
- Cause 10
References
Differentiating Autoimmune lymphoproliferative syndrome from other Diseases
Overview
[Disease name] must be differentiated from other diseases that cause [clinical feature 1], [clinical feature 2], and [clinical feature 3], such as [differential dx1], [differential dx2], and [differential dx3].
OR
[Disease name] must be differentiated from [[differential dx1], [differential dx2], and [differential dx3].
Differentiating [Disease name] from other Diseases
[Disease name] must be differentiated from other diseases that cause [clinical feature 1], [clinical feature 2], and [clinical feature 3], such as [differential dx1], [differential dx2], and [differential dx3].
OR
[Disease name] must be differentiated from [differential dx1], [differential dx2], and [differential dx3].
OR
As [disease name] manifests in a variety of clinical forms, differentiation must be established in accordance with the particular subtype. [Subtype name 1] must be differentiated from other diseases that cause [clinical feature 1], such as [differential dx1] and [differential dx2]. In contrast, [subtype name 2] must be differentiated from other diseases that cause [clinical feature 2], such as [differential dx3] and [differential dx4].
Differentiating [disease name] from other diseases on the basis of [symptom 1], [symptom 2], and [symptom 3]
On the basis [symptom 1], [symptom 2], and [symptom 3], [disease name] must be differentiated from [disease 1], [disease 2], [disease 3], [disease 4], [disease 5], and [disease 6].
| Diseases | Clinical manifestations | Para-clinical findings | Gold standard | Additional findings | |||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Symptoms | Physical examination | ||||||||||||||
| Lab Findings | Imaging | Histopathology | |||||||||||||
| Symptom 1 | Symptom 2 | Symptom 3 | Physical exam 1 | Physical exam 2 | Physical exam 3 | Lab 1 | Lab 2 | Lab 3 | Imaging 1 | Imaging 2 | Imaging 3 | ||||
| Differential Diagnosis 1 | |||||||||||||||
| Differential Diagnosis 2 | |||||||||||||||
| Differential Diagnosis 3 | |||||||||||||||
| Diseases | Symptom 1 | Symptom 2 | Symptom 3 | Physical exam 1 | Physical exam 2 | Physical exam 3 | Lab 1 | Lab 2 | Lab 3 | Imaging 1 | Imaging 2 | Imaging 3 | Histopathology | Gold standard | Additional findings |
| Differential Diagnosis 4 | |||||||||||||||
| Differential Diagnosis 5 | |||||||||||||||
| Differential Diagnosis 6 | |||||||||||||||
References
Epidemiology and Demographics
Overview
Epidemiology and Demographics
Incidence
- The incidence/prevalence of [disease name] is approximately [number range] per 100,000 individuals worldwide.
- In [year], the incidence/prevalence of [disease name] was estimated to be [number range] cases per 100,000 individuals worldwide.
Prevalence
- The incidence/prevalence of [disease name] is approximately [number range] per 100,000 individuals worldwide.
- In [year], the incidence/prevalence of [disease name] was estimated to be [number range] cases per 100,000 individuals worldwide.
- The prevalence of [disease/malignancy] is estimated to be [number] cases annually.
Case-fatality rate/Mortality rate
- In [year], the incidence of [disease name] is approximately [number range] per 100,000 individuals with a case-fatality rate/mortality rate of [number range]%.
- The case-fatality rate/mortality rate of [disease name] is approximately [number range].
Age
- Patients of all age groups may develop [disease name].
- The incidence of [disease name] increases with age; the median age at diagnosis is [#] years.
- [Disease name] commonly affects individuals younger than/older than [number of years] years of age.
- [Chronic disease name] is usually first diagnosed among [age group].
- [Acute disease name] commonly affects [age group].
Race
- There is no racial predilection to [disease name].
- [Disease name] usually affects individuals of the [race 1] race. [Race 2] individuals are less likely to develop [disease name].
Gender
- [Disease name] affects men and women equally.
- [Gender 1] are more commonly affected by [disease name] than [gender 2]. The [gender 1] to [gender 2] ratio is approximately [number > 1] to 1.
Region
- The majority of [disease name] cases are reported in [geographical region].
- [Disease name] is a common/rare disease that tends to affect [patient population 1] and [patient population 2].
Developed Countries
Developing Countries
References
Risk Factors
Overview
There are no established risk factors for [disease name].
OR
The most potent risk factor in the development of [disease name] is [risk factor 1]. Other risk factors include [risk factor 2], [risk factor 3], and [risk factor 4].
OR
Common risk factors in the development of [disease name] include [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].
OR
Common risk factors in the development of [disease name] may be occupational, environmental, genetic, and viral.
Risk Factors
There are no established risk factors for [disease name].
OR
The most potent risk factor in the development of [disease name] is [risk factor 1]. Other risk factors include [risk factor 2], [risk factor 3], and [risk factor 4].
OR
Common risk factors in the development of [disease name] include [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].
Common Risk Factors
- Common risk factors in the development of [disease name] may be occupational, environmental, genetic, and viral.
- Common risk factors in the development of [disease name] include:
- [Risk factor 1]
- [Risk factor 2]
- [Risk factor 3]
Less Common Risk Factors
- Less common risk factors in the development of [disease name] include:
- [Risk factor 1]
- [Risk factor 2]
- [Risk factor 3]
References
Screening
Overview
There is insufficient evidence to recommend routine screening for [disease/malignancy].
OR
According to the [guideline name], screening for [disease name] is not recommended.
OR
According to the [guideline name], screening for [disease name] by [test 1] is recommended every [duration] among patients with [condition 1], [condition 2], and [condition 3].
Screening
There is insufficient evidence to recommend routine screening for [disease/malignancy].
OR
According to the [guideline name], screening for [disease name] is not recommended.
OR
According to the [guideline name], screening for [disease name] by [test 1] is recommended every [duration] among patients with:
- [Condition 1]
- [Condition 2]
- [Condition 3]
References
Natural History, Complications and Prognosis
Overview
If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
OR
Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
OR
Prognosis is generally excellent/good/poor, and the 1/5/10-year mortality/survival rate of patients with [disease name] is approximately [#]%.
Natural History, Complications, and Prognosis
Natural History
- The symptoms of (disease name) usually develop in the first/ second/ third decade of life, and start with symptoms such as ___.
- The symptoms of (disease name) typically develop ___ years after exposure to ___.
- If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
Complications
- Common complications of [disease name] include:
- [Complication 1]
- [Complication 2]
- [Complication 3]
Prognosis
- Prognosis is generally excellent/good/poor, and the 1/5/10-year mortality/survival rate of patients with [disease name] is approximately [--]%.
- Depending on the extent of the [tumor/disease progression] at the time of diagnosis, the prognosis may vary. However, the prognosis is generally regarded as poor/good/excellent.
- The presence of [characteristic of disease] is associated with a particularly [good/poor] prognosis among patients with [disease/malignancy].
- [Subtype of disease/malignancy] is associated with the most favorable prognosis.
- The prognosis varies with the [characteristic] of tumor; [subtype of disease/malignancy] have the most favorable prognosis.
References
Diagnosis
Diagnostic Criteria | History and Symptoms | Physical Examination | Laboratory Findings | Electrocardiogram | 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