Autoimmune lymphoproliferative syndrome classification: Difference between revisions
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==Overview== | ==Overview== | ||
[[Classification]] of [[ALPS]] is based on the recommendations made by first international [[ALPS]] workshop held at National Institutes of Health in 2009. | |||
==Classification== | ==Classification== | ||
Revision as of 02:31, 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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Diagnosis |
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Treatment |
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Autoimmune lymphoproliferative syndrome classification On the Web |
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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 of ALPS is based on the recommendations made by first international ALPS workshop held at National Institutes of Health in 2009.
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