Autoimmune lymphoproliferative syndrome classification: Difference between revisions
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! | The revised [[classification]] of [[ALPS]] is as following | ||
! | !Previous nomenclature | ||
! | !Revised nomenclature | ||
! | !Gene | ||
!Definition | |||
|- | |- | ||
| | |[[ALPS]]type IIb | ||
| | |CEDS | ||
| | |CASP8 | ||
| | |[[Splenomegaly]], marginal raised DNT, recurrent [[infections]], [[germline]] [[mutations]] in [[caspase]] 8 | ||
|- | |- | ||
| | |[[ALPS]] type IV | ||
| | |RALD | ||
| | |NRAS | ||
| | |[[Autoimmunity]], [[lymphadenopathy]] and/or [[splenomegaly]], elevated or normal DNTs, [[somatic]] mutations in NRAS | ||
|- | |- | ||
| | |DALD | ||
| | |DALD | ||
| | |Unknown | ||
| | |[[Lymphadenopathy]] and /or [[splenomegaly]], autoimmunity, normal DNTs, defective in vitro FAS-mediated apoptosis | ||
|- | |||
|XLP1 | |||
|XLP1 | |||
|SH2D1A | |||
|[[Hypogammaglobulinemia]], fulminant Epstein- Barr virus [[infection]], or [[lymphoma]] | |||
|} | |} | ||
<br /> | |||
==References== | ==References== | ||
Revision as of 04:09, 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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Case Studies |
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Autoimmune lymphoproliferative syndrome classification On the Web |
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American Roentgen Ray Society Images of Autoimmune lymphoproliferative syndrome classification |
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FDA on Autoimmune lymphoproliferative syndrome classification |
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CDC on Autoimmune lymphoproliferative syndrome classification |
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Autoimmune lymphoproliferative syndrome classification in the news |
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Blogs on Autoimmune lymphoproliferative syndrome classification |
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Directions to Hospitals Treating Autoimmune lymphoproliferative syndrome |
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Risk calculators and risk factors for Autoimmune lymphoproliferative syndrome classification |
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
| Previous nomenclature | Revised nomenclature | Gene | Definition |
|---|---|---|---|
| ALPStype IIb | CEDS | CASP8 | Splenomegaly, marginal raised DNT, recurrent infections, germline mutations in caspase 8 |
| ALPS type IV | RALD | NRAS | Autoimmunity, lymphadenopathy and/or splenomegaly, elevated or normal DNTs, somatic mutations in NRAS |
| DALD | DALD | Unknown | Lymphadenopathy and /or splenomegaly, autoimmunity, normal DNTs, defective in vitro FAS-mediated apoptosis |
| XLP1 | XLP1 | SH2D1A | Hypogammaglobulinemia, fulminant Epstein- Barr virus infection, or lymphoma |