Autoimmune lymphoproliferative syndrome: Difference between revisions

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{{Autoimmune lymphoproliferative syndrome}}
{{Autoimmune lymphoproliferative syndrome}}


'''Editor-In-Chief:''' David Teachey, MD [mailto:TEACHEYD@email.chop.edu]
'''Editor-In-Chief:''' David Teachey, MD [mailto:TEACHEYD@email.chop.edu] '''Associate editor in chief''': {{SharmiB}}


{{SK}} Canale-Smith syndrome; ALPS
{{SK}} Canale-Smith syndrome; ALPS
Line 25: Line 25:


==[[Autoimmune lymphoproliferative syndrome historical perspective|Historical Perspective]]==
==[[Autoimmune lymphoproliferative syndrome historical perspective|Historical Perspective]]==
==== Overview[edit | edit source] ====
==== Historical Perspective[edit | edit source] ====
==== References ====
==Discovery==
<br />
== 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 ==


==[[Autoimmune lymphoproliferative syndrome classification|Classification]]==
==[[Autoimmune lymphoproliferative syndrome classification|Classification]]==
<br />
== 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 ==
==[[Autoimmune lymphoproliferative syndrome pathophysiology|Pathophysiology]]==
==[[Autoimmune lymphoproliferative syndrome pathophysiology|Pathophysiology]]==
<br />
== 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 ==
==[[Autoimmune lymphoproliferative syndrome causes|Causes]]==
==[[Autoimmune lymphoproliferative syndrome causes|Causes]]==
<br />
== 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 ==
==[[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 />
== 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].
{| class="wikitable"
! rowspan="4" |Diseases
| colspan="6" rowspan="1" |'''Clinical manifestations'''
! colspan="7" rowspan="2" |Para-clinical findings
| colspan="1" rowspan="4" |'''Gold standard'''
! rowspan="4" |Additional findings
|-
| colspan="3" rowspan="2" |'''Symptoms'''
! colspan="3" rowspan="2" |Physical examination
|-
! colspan="3" |Lab Findings
! colspan="3" |Imaging
! rowspan="2" |Histopathology
|-
!Symptom 1
! colspan="1" rowspan="1" |Symptom 2
!Symptom 3
!Physical exam 1
! colspan="1" rowspan="1" |Physical exam 2
!Physical exam 3
!Lab 1
!Lab 2
!Lab 3
!Imaging 1
!Imaging 2
!Imaging 3
|-
|Differential Diagnosis 1
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|Differential Diagnosis 2
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|Differential Diagnosis 3
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!Diseases
!Symptom 1
! colspan="1" rowspan="1" |Symptom 2
!Symptom 3
!Physical exam 1
! colspan="1" rowspan="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
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|Differential Diagnosis 5
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|Differential Diagnosis 6
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|}
== References ==
==[[Autoimmune lymphoproliferative syndrome epidemiology and demographics|Epidemiology and Demographics]]==
==[[Autoimmune lymphoproliferative syndrome epidemiology and demographics|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 ==


==[[Autoimmune lymphoproliferative syndrome risk factors|Risk Factors]]==
==[[Autoimmune lymphoproliferative syndrome risk factors|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 ==


==[[Autoimmune lymphoproliferative syndrome screening|Screening]]==
==[[Autoimmune lymphoproliferative syndrome screening|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 ==


==[[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 ==
==References==
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==
==Diagnosis==

Latest revision as of 04:06, 3 August 2021

Autoimmune lymphoproliferative syndrome
OMIM 601859 603909
DiseasesDB 33425 Template:DiseasesDB2

Autoimmune lymphoproliferative syndrome Microchapters

Home

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Autoimmune lymphoproliferative syndrome from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

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

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Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

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Risk calculators and risk factors for Autoimmune lymphoproliferative syndrome

Editor-In-Chief: David Teachey, MD [1] Associate editor in chief: Sharmi Biswas, M.B.B.S

Synonyms and keywords: Canale-Smith syndrome; ALPS

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Autoimmune lymphoproliferative syndrome from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and 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

Case Studies

Case #1

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