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| {{Autoimmune lymphoproliferative syndrome}} | | {{Autoimmune lymphoproliferative syndrome}} |
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| '''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}} |
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| {{SK}} Canale-Smith syndrome; ALPS | | {{SK}} Canale-Smith syndrome; ALPS |
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| ==[[Autoimmune lymphoproliferative syndrome historical perspective|Historical Perspective]]== | | ==[[Autoimmune lymphoproliferative syndrome historical perspective|Historical Perspective]]== |
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| ==== Overview[edit | edit source] ====
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| ==== Historical Perspective[edit | edit source] ====
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| ==== References ====
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| ==Discovery==
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| <br />
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| == Overview ==
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| The exact pathogenesis of [disease name] is not fully understood.
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| OR
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| 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].
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|
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| OR
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| [Pathogen name] is usually transmitted via the [transmission route] route to the human host.
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| OR
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| Following transmission/ingestion, the [pathogen] uses the [entry site] to invade the [cell name] cell.
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| OR
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| [Disease or malignancy name] arises from [cell name]s, which are [cell type] cells that are normally involved in [function of cells].
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| OR
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| The progression to [disease name] usually involves the [molecular pathway].
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| OR
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| The pathophysiology of [disease/malignancy] depends on the histological subtype.
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| == Pathophysiology ==
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| === Physiology ===
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| The normal physiology of [name of process] can be understood as follows:
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| === Pathogenesis ===
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|
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| * The exact pathogenesis of [disease name] is not completely understood.
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|
| |
| OR
| |
|
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| * 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].
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| * [Pathogen name] is usually transmitted via the [transmission route] route to the human host.
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| * Following transmission/ingestion, the [pathogen] uses the [entry site] to invade the [cell name] cell.
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| * [Disease or malignancy name] arises from [cell name]s, which are [cell type] cells that are normally involved in [function of cells].
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| * The progression to [disease name] usually involves the [molecular pathway].
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| * The pathophysiology of [disease/malignancy] depends on the histological subtype.
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|
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| == Genetics ==
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| [Disease name] is transmitted in [mode of genetic transmission] pattern.
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| OR
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| Genes involved in the pathogenesis of [disease name] include:
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| * [Gene1]
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| * [Gene2]
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| * [Gene3]
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| OR
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| The development of [disease name] is the result of multiple genetic mutations such as:
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|
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| * [Mutation 1]
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| * [Mutation 2]
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| * [Mutation 3]
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| == Associated Conditions ==
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| Conditions associated with [disease name] include:
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|
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| * [Condition 1]
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| * [Condition 2]
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| * [Condition 3]
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| == Gross Pathology ==
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| On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
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| == Microscopic Pathology ==
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| On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
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| == References ==
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| ==[[Autoimmune lymphoproliferative syndrome classification|Classification]]== | | ==[[Autoimmune lymphoproliferative syndrome classification|Classification]]== |
| <br />
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| == Overview ==
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| There is no established system for the classification of [disease name].
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| OR
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| [Disease name] may be classified according to [classification method] into [number] subtypes/groups: [group1], [group2], [group3], and [group4].
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| OR
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| [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].
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| OR
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| Based on the duration of symptoms, [disease name] may be classified as either acute or chronic.
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| OR
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| 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].
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| OR
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| The staging of [malignancy name] is based on the [staging system].
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| OR
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| There is no established system for the staging of [malignancy name].
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|
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| == Classification ==
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| There is no established system for the classification of [disease name].
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|
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| OR
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|
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| [Disease name] may be classified according to [classification method] into [number] subtypes/groups:
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|
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| * [Group1]
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| * [Group2]
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| * [Group3]
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| * [Group4]
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| OR
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|
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| [Disease name] may be classified into [large number > 6] subtypes based on:
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|
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| * [Classification method 1]
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| * [Classification method 2]
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| * [Classification method 3]
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|
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| [Disease name] may be classified into several subtypes based on:
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|
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| * [Classification method 1]
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| * [Classification method 2]
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| * [Classification method 3]
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| OR
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| Based on the duration of symptoms, [disease name] may be classified as either acute or chronic.
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|
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| OR
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|
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| '''If the staging system involves specific and characteristic findings and features:'''
| |
|
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| 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].
| |
|
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| OR
| |
|
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| The staging of [malignancy name] is based on the [staging system].
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|
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| OR
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|
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| There is no established system for the staging of [malignancy name].
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|
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| == References ==
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| ==[[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 ==
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|
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| ==[[Autoimmune lymphoproliferative syndrome causes|Causes]]== | | ==[[Autoimmune lymphoproliferative syndrome causes|Causes]]== |
| <br />
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|
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| == Overview[edit | edit source] ==
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| Disease name] may be caused by [cause1], [cause2], or [cause3].
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| OR
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| Common causes of [disease] include [cause1], [cause2], and [cause3].
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| OR
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| The most common cause of [disease name] is [cause 1]. Less common causes of [disease name] include [cause 2], [cause 3], and [cause 4].
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| OR
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| The cause of [disease name] has not been identified. To review risk factors for the development of [disease name], click here.
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| == Causes[edit | edit source] ==
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| * ymptom/manifestation] include [cause1], [cause2], and [cause3].
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| * [Cause] is a life-threatening cause of [disease].
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|
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| === Common Causes[edit | edit source] ===
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| Common causes of [disease name] may include:
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|
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| * [Cause1]
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| * [Cause2]
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| * [Cause3]
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|
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| OR
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| * [Disease name] is caused by an infection with [pathogen name].
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| * [Pathogen name] is caused by [pathogen name].
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|
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| === Less Common Causes[edit | edit source] ===
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| Less common causes of [disease name] include:
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|
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| * [Cause1]
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| * [Cause2]
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| * [CauseCauses by OrganList the causes of the disease in alphabetical order:
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| * Cause 1
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| * Cause 2
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| * Cause 3
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| * Cause 4
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| * Cause 5
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| * Cause 6
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| * Cause 7
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| * Cause 8
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| * Cause 9
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| * Cause 10
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|
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| == References ==
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| ==[[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 />
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|
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| == Overview ==
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| [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].
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| OR
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| [Disease name] must be differentiated from [[differential dx1], [differential dx2], and [differential dx3].
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|
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| == Differentiating [Disease name] from other Diseases ==
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| [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].
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|
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| OR
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|
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| [Disease name] must be differentiated from [differential dx1], [differential dx2], and [differential dx3].
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| OR
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|
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| 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].
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|
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| === Differentiating [disease name] from other diseases on the basis of [symptom 1], [symptom 2], and [symptom 3] ===
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| 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].
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| {| class="wikitable"
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| ! rowspan="4" |Diseases
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| | colspan="6" rowspan="1" |'''Clinical manifestations'''
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| ! colspan="7" rowspan="2" |Para-clinical findings
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| | colspan="1" rowspan="4" |'''Gold standard'''
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| ! rowspan="4" |Additional findings
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| |-
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| | colspan="3" rowspan="2" |'''Symptoms'''
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| ! colspan="3" rowspan="2" |Physical examination
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| |-
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| ! colspan="3" |Lab Findings
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| ! colspan="3" |Imaging
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| ! rowspan="2" |Histopathology
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| |-
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| !Symptom 1
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| ! colspan="1" rowspan="1" |Symptom 2
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| !Symptom 3
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| !Physical exam 1
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| ! colspan="1" rowspan="1" |Physical exam 2
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| !Physical exam 3
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| !Lab 1
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| !Lab 2
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| !Lab 3
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| !Imaging 1
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| !Imaging 2
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| !Imaging 3
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| |-
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| |Differential Diagnosis 1
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| |-
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| |Differential Diagnosis 2
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| |-
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| |Differential Diagnosis 3
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| |-
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| !Diseases
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| !Symptom 1
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| ! colspan="1" rowspan="1" |Symptom 2
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| !Symptom 3
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| !Physical exam 1
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| ! colspan="1" rowspan="1" |Physical exam 2
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| !Physical exam 3
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| !Lab 1
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| !Lab 2
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| !Lab 3
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| !Imaging 1
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| !Imaging 2
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| !Imaging 3
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| !Histopathology
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| |'''Gold standard'''
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| !Additional findings
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| |-
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| |Differential Diagnosis 4
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| |-
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| |Differential Diagnosis 5
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| |-
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| |Differential Diagnosis 6
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| |}
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| == References ==
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| ==[[Autoimmune lymphoproliferative syndrome epidemiology and demographics|Epidemiology and Demographics]]== | | ==[[Autoimmune lymphoproliferative syndrome epidemiology and demographics|Epidemiology and Demographics]]== |
|
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| == Overview ==
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|
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| == Epidemiology and Demographics ==
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|
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| === Incidence ===
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|
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| * The incidence/prevalence of [disease name] is approximately [number range] per 100,000 individuals worldwide.
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| * In [year], the incidence/prevalence of [disease name] was estimated to be [number range] cases per 100,000 individuals worldwide.
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|
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| === 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.
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| * The prevalence of [disease/malignancy] is estimated to be [number] cases annually.
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|
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| === Case-fatality rate/Mortality rate ===
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|
| |
| * 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]%.
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| * The case-fatality rate/mortality rate of [disease name] is approximately [number range].
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|
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| === Age ===
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|
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| * Patients of all age groups may develop [disease name].
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| * The incidence of [disease name] increases with age; the median age at diagnosis is [#] years.
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| * [Disease name] commonly affects individuals younger than/older than [number of years] years of age.
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| * [Chronic disease name] is usually first diagnosed among [age group].
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| * [Acute disease name] commonly affects [age group].
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|
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| === Race ===
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|
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| * There is no racial predilection to [disease name].
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| * [Disease name] usually affects individuals of the [race 1] race. [Race 2] individuals are less likely to develop [disease name].
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|
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| === Gender ===
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|
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| * [Disease name] affects men and women equally.
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| * [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.
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|
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| === Region ===
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|
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| * The majority of [disease name] cases are reported in [geographical region].
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|
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| * [Disease name] is a common/rare disease that tends to affect [patient population 1] and [patient population 2].
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|
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| === Developed Countries ===
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| === Developing Countries ===
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| == References ==
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|
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|
| ==[[Autoimmune lymphoproliferative syndrome risk factors|Risk Factors]]== | | ==[[Autoimmune lymphoproliferative syndrome risk factors|Risk Factors]]== |
|
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| == Overview ==
| |
| There are no established risk factors for [disease name].
| |
|
| |
| OR
| |
|
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| 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].
| |
|
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| OR
| |
|
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| Common risk factors in the development of [disease name] include [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].
| |
|
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| OR
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|
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| 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 ==
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| There is insufficient evidence to recommend routine screening for [disease/malignancy].
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| OR
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| According to the [guideline name], screening for [disease name] is not recommended.
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| OR
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| 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].
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| == Screening ==
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| There is insufficient evidence to recommend routine screening for [disease/malignancy].
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| OR
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| According to the [guideline name], screening for [disease name] is not recommended.
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| OR
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| According to the [guideline name], screening for [disease name] by [test 1] is recommended every [duration] among patients with:
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| * [Condition 1]
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| * [Condition 2]
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| * [Condition 3]
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| == References ==
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| ==[[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]]== |
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| == Overview ==
| | ==References== |
| If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
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| OR
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| Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
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| OR
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| Prognosis is generally excellent/good/poor, and the 1/5/10-year mortality/survival rate of patients with [disease name] is approximately [#]%.
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| == Natural History, Complications, and Prognosis ==
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| === Natural History ===
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| * The symptoms of (disease name) usually develop in the first/ second/ third decade of life, and start with symptoms such as ___.
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| * The symptoms of (disease name) typically develop ___ years after exposure to ___.
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| * If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
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| === Complications ===
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| * Common complications of [disease name] include:
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| ** [Complication 1]
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| ** [Complication 2]
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| ** [Complication 3]
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| === Prognosis ===
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| * Prognosis is generally excellent/good/poor, and the 1/5/10-year mortality/survival rate of patients with [disease name] is approximately [--]%.
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| * 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.
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| * The presence of [characteristic of disease] is associated with a particularly [good/poor] prognosis among patients with [disease/malignancy].
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| * [Subtype of disease/malignancy] is associated with the most favorable prognosis.
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| * The prognosis varies with the [characteristic] of tumor; [subtype of disease/malignancy] have the most favorable prognosis.
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| == References == | |
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| ==Diagnosis== | | ==Diagnosis== |