Autoimmune lymphoproliferative syndrome classification: Difference between revisions
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==Classification== | ==Classification== | ||
The | The presence of 2 required and 6 additional [[criteria]] are required to [[diagnose]] [[Autoimmune lymphoproliferative syndrome]]([[ALPS]]). | ||
Required [[criteria]]- | |||
* [[Chronic]] [[lymphadenopathy]] | |||
* [[Splenomegaly]] (+/-) | |||
* Increased circulating TCRαβ+ DNT [[cells]] | |||
Additional criteria- | |||
* Primary | |||
- Abnormal [[lymphocyte]] [[apoptosis]] assay | |||
- Presence of [[pathogenic]] [[mutations]] in FAS pathway [[genes]] | |||
* Secondary- | |||
- Elevated circulating [[biomarkers]] | |||
- characteristic [[histopathology]] of [[ALPS]] | |||
- Family history of [[ALPS]] | |||
Definitive [[diagnosis]] of [[ALPS]]- 2 required [[criteria]] and either of the 2 primary additional [[criteria]] | |||
Probable [[diagnosis]]- 2 required [[criteria]] and one of the primary additional [[criteria]] | |||
==References== | ==References== | ||
Revision as of 02:29, 6 August 2021
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Autoimmune lymphoproliferative syndrome Microchapters |
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Differentiating Autoimmune lymphoproliferative syndrome from other Diseases |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: David Teachey, MD [2] Sharmi Biswas, M.B.B.S
Overview
Classification
The presence of 2 required and 6 additional criteria are required to diagnose Autoimmune lymphoproliferative syndrome(ALPS).
Required criteria-
- Splenomegaly (+/-)
- Increased circulating TCRαβ+ DNT cells
Additional criteria-
- Primary
- Abnormal lymphocyte apoptosis assay
- Presence of pathogenic mutations in FAS pathway genes
- Secondary-
- Elevated circulating biomarkers
- characteristic histopathology of ALPS
- Family history of ALPS
Definitive diagnosis of ALPS- 2 required criteria and either of the 2 primary additional criteria
Probable diagnosis- 2 required criteria and one of the primary additional criteria