Autoimmune lymphoproliferative syndrome laboratory findings: Difference between revisions
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{{Autoimmune lymphoproliferative syndrome}} | {{Autoimmune lymphoproliferative syndrome}} | ||
'''Editor-In-Chief:''' David Teachey, MD [mailto:TEACHEYD@email.chop.edu] | '''Editor-In-Chief:''' {{CMG}} David Teachey, MD [mailto:TEACHEYD@email.chop.edu] {{AE}}{{SharmiB}} | ||
==Overview== | ==Overview== | ||
An elevated concentration of [[serum]] double negative α/β T cells comprising more than 1.5% of the total [[lymphocytes]] or at least 2.5% of total [[T cells]] along with [[chronic]] [[lymphadenopathy]] or [[splenomegaly]] for more than 6 months are the two required testing for clinical [[diagnosis]] of [[Autoimmune lymphoproliferative syndrome]] which leads to ancillary testing. Confirmatory testing for ALPS is the testing for the [[ALPS]]-related [[mutations]] or functional testing of patient T cells are requires according to the 2010 guidelines. | |||
==Laboratory Findings== | ==Laboratory Findings== | ||
In [[peripheral blood]]-<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><ref name="LiHuang2015">{{cite journal|last1=Li|first1=Pu|last2=Huang|first2=Ping|last3=Yang|first3=Ye|last4=Hao|first4=Mu|last5=Peng|first5=Hongwei|last6=Li|first6=Fei|title=Updated Understanding of Autoimmune Lymphoproliferative Syndrome (ALPS)|journal=Clinical Reviews in Allergy & Immunology|volume=50|issue=1|year=2015|pages=55–63|issn=1080-0549|doi=10.1007/s12016-015-8466-y}}</ref> | |||
* Increased [[T cell]] [[receptor]] (TCR) αβ+/CD4-/CD8- [[T cells]] | |||
* Increased [[lymphoid]] [[tissue]] | |||
* Co-expression of CD45RA,CD57,CD27,CD28, [[perforin]], and [[HLA-DR]] in the DNT [[cells]] but lacking CD45R0 and CD56 | |||
* Cytopenia | |||
* Positive Coombs' test | |||
* Increased soluble FAS ligand (FasL) | |||
* High vitamin B12 | |||
* Hypergammaglobulinemia ( increased IgG, IgA,or IgM) | |||
* | |||
* | |||
==References== | ==References== | ||
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[[Category:Disease]] | [[Category:Disease]] | ||
[[Category:Hematology]] | [[Category:Hematology]] | ||
Latest revision as of 02:37, 11 August 2021
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Editor-In-Chief: Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] David Teachey, MD [2] Associate Editor(s)-in-Chief: Sharmi Biswas, M.B.B.S
Overview
An elevated concentration of serum double negative α/β T cells comprising more than 1.5% of the total lymphocytes or at least 2.5% of total T cells along with chronic lymphadenopathy or splenomegaly for more than 6 months are the two required testing for clinical diagnosis of Autoimmune lymphoproliferative syndrome which leads to ancillary testing. Confirmatory testing for ALPS is the testing for the ALPS-related mutations or functional testing of patient T cells are requires according to the 2010 guidelines.
Laboratory Findings
- Co-expression of CD45RA,CD57,CD27,CD28, perforin, and HLA-DR in the DNT cells but lacking CD45R0 and CD56
- Cytopenia
- Positive Coombs' test
- Increased soluble FAS ligand (FasL)
- High vitamin B12
- Hypergammaglobulinemia ( increased IgG, IgA,or IgM)
References
- ↑ 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.
- ↑ Li, Pu; Huang, Ping; Yang, Ye; Hao, Mu; Peng, Hongwei; Li, Fei (2015). "Updated Understanding of Autoimmune Lymphoproliferative Syndrome (ALPS)". Clinical Reviews in Allergy & Immunology. 50 (1): 55–63. doi:10.1007/s12016-015-8466-y. ISSN 1080-0549.