Autoimmune lymphoproliferative syndrome classification: Difference between revisions
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==Classification== | ==Classification== | ||
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. | |||
==References== | ==References== | ||
Revision as of 02:10, 6 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
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.