Autoimmune lymphoproliferative syndrome classification: Difference between revisions

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==Classification==
==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].
Old nomenclature<ref name="pmid12819469">{{cite journal| author=Sneller MC, Dale JK, Straus SE| title=Autoimmune lymphoproliferative syndrome. | journal=Curr Opin Rheumatol | year= 2003 | volume= 15 | issue= 4 | pages= 417-21 | pmid=12819469 | doi= | pmc= | url= }} </ref>
Old nomenclature<ref name="pmid12819469">{{cite journal| author=Sneller MC, Dale JK, Straus SE| title=Autoimmune lymphoproliferative syndrome. | journal=Curr Opin Rheumatol | year= 2003 | volume= 15 | issue= 4 | pages= 417-21 | pmid=12819469 | doi= | pmc= | url= }} </ref>
* IA - [[Fas]]  
* IA - [[Fas]]  

Revision as of 14:10, 22 June 2021

Autoimmune lymphoproliferative syndrome Microchapters

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; David Teachey, MD [2]

Overview

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]. Old nomenclature[1]

Revised nomenclature (2010)[2]

  • ALPS-FAS: Fas. Germline FAS mutations. 70% of patients. Autosomal dominant. Dominant negative and haploinsufficient mutations described.[3]
  • ALPS-sFAS: Fas. Somatic FAS mutations in DNT compartment.[4] 10% of patients
  • ALPS-FASL: Fas ligand. Germline FASL mutations. 3 reported cases
  • ALPS-CASP10: Caspase 10. Germline CASP10 mutation. 2% of patients
  • ALPS-U: Undefined. 20% of patients
  • CEDS: Caspase 8 deficiency state. No longer considered a subtype of ALPS but distinct disorder
  • RALD: NRAS , KRAS. Somatic mutations in NRAS and KRAS in lympocyte comparment. No longer considered a subtype of ALPS but distinct disesase.

References

  1. Sneller MC, Dale JK, Straus SE (2003). "Autoimmune lymphoproliferative syndrome". Curr Opin Rheumatol. 15 (4): 417–21. PMID 12819469.
  2. Oliveira JB, Bleesing JJ, Dianzani U, Fleisher TA, Jaffe ES, Lenardo MJ; et al. (2010). "Revised diagnostic criteria and classification for the autoimmune lymphoproliferative syndrome (ALPS): report from the 2009 NIH International Workshop". Blood. 116 (14): e35–40. doi:10.1182/blood-2010-04-280347. PMC 2953894. PMID 20538792.
  3. Kuehn HS, Caminha I, Niemela JE, Rao VK, Davis J, Fleisher TA; et al. (2011). "FAS haploinsufficiency is a common disease mechanism in the human autoimmune lymphoproliferative syndrome". J Immunol. 186 (10): 6035–43. doi:10.4049/jimmunol.1100021. PMID 21490157.
  4. Holzelova E, Vonarbourg C, Stolzenberg MC, Arkwright PD, Selz F, Prieur AM; et al. (2004). "Autoimmune lymphoproliferative syndrome with somatic Fas mutations". N Engl J Med. 351 (14): 1409–18. doi:10.1056/NEJMoa040036. PMID 15459302.

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