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==
 
{| class="wikitable"
The diagnosis of ALPS is currently based on the presence of 2 required and 6 additional criteria. Required criteria include the presence of chronic lymphadenopathy and/or splenomegaly and elevated circulating TCRαβ+ DNT cells. Additional criteria are further divided into primary and secondary categories (Table 2). An abnormal lymphocyte apoptosis assay and the presence of pathogenic mutations in genes of the FAS pathway are the primary additional criteria. Secondary additional criteria consist of elevated circulating biomarkers, characteristic histopathology, and family history compatible with ALPS. For a definitive ALPS diagnosis, a patient has to meet both required criteria and either of the 2 primary additional criteria (Table 2). A probable ALPS diagnosis can be entertained if both the required criteria and any one of the secondary additional criteria 3, 4, 5, or 6 are present. Patients with probable ALPS should be treated and monitored in the same way as patients with a definitive diagnosis, but treating physicians are advised to pursue a genetic or apoptosis assay-based diagnostic workup whenever possible.
|+
The revised [[classification]] of [[ALPS]] is as following <ref name="OliveiraBleesing2010">{{cite journal|last1=Oliveira|first1=Joao B.|last2=Bleesing|first2=Jack J.|last3=Dianzani|first3=Umberto|last4=Fleisher|first4=Thomas A.|last5=Jaffe|first5=Elaine S.|last6=Lenardo|first6=Michael J.|last7=Rieux-Laucat|first7=Frederic|last8=Siegel|first8=Richard M.|last9=Su|first9=Helen C.|last10=Teachey|first10=David T.|last11=Rao|first11=V. Koneti|title=Revised diagnostic criteria and classification for the autoimmune lymphoproliferative syndrome (ALPS): report from the 2009 NIH International Workshop|journal=Blood|volume=116|issue=14|year=2010|pages=e35–e40|issn=0006-4971|doi=10.1182/blood-2010-04-280347}}</ref>
!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==
{{reflist|2}}
{{reflist|2}}


[[Category:Hematology]]
[[Category:Hematology]]

Latest revision as of 02:19, 11 August 2021

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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 revised classification of ALPS is as following [1]
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


References

  1. ↑ Oliveira, Joao B.; Bleesing, Jack J.; Dianzani, Umberto; Fleisher, Thomas A.; Jaffe, Elaine S.; Lenardo, Michael J.; Rieux-Laucat, Frederic; Siegel, Richard M.; Su, Helen C.; Teachey, David T.; Rao, V. Koneti (2010). "Revised diagnostic criteria and classification for the autoimmune lymphoproliferative syndrome (ALPS): report from the 2009 NIH International Workshop". Blood. 116 (14): e35–e40. doi:10.1182/blood-2010-04-280347. ISSN 0006-4971.
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