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. 2024 Jul 12;15:1402038. doi: 10.3389/fimmu.2024.1402038

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Copyright © 2024 Tahiat, Belbouab, Yagoubi, Hakem, Fernini, Keddari, Belhadj, Touri, Aggoune, Stoddard, Niemela, Zerifi, Melzi, Aboura, Saad-Djaballah, Ferhani, Ketfi, Messaoudi, Bencharif Madani, Benhacine, Dehimi, Okka, Amroune, Fellahi, Bendahmane, Khoulani, Oukil, Soufane, Bourelaf, Boubidi, Boukhenfouf, Amine Ifri, Khelafi, Boudiaf, Khelifi Touhami, Meçabih, Boucelma, Zelaci, Gacem, Ladj, Mekki, Bensaadi, Benhalima, Zeroual, Bioud, Benameur, Bouhdjila, Bouzerar, Ibsaine, Maouche, Kedji, Smati, Boukari, Lambert, Rosenzweig, Notarangelo and Djenouhat

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Flow cytometric workup based on the clinical presentation. (A), FCM based strategy in the context of nonspecific IEI manifestations. (B), diagnostic strategy in the context of specific clinical presentations. T1 panel, CD3, CD4, CD8, CD45RA, CD45RO, CCR7; T3 panel, CD3, CD4, CD45RO, CXCR3, CCR6, CXCR5; T4 panel, TCRαβ, TCRγδ, CD3, CD4, CD8, CD45; B panel, CD19, CD27, sIgD, CD38, CD24, CD21. ALPS, autoimmune lymphoproliferative syndrome; AR, autosomal recessive; CGD, chronic granulomatous disease; CHAPLE, complement hyperactivation angiopathic thrombosis and protein-losing enteropathy; CHS, Chediak-Higashi syndrome; CID, combined immunodeficiency; CVID, common variable immunodeficiency; DHR, Dihydrorhodamine; GS2, Griscelli syndrome type 2; HIES, hyper-IgE syndrome; HIMS, hyper-IgM syndrome; LAD, leukocyte adhesion deficiency; LRBA, LPS-responsive beige-like anchor protein; SCID, severe combined immunodeficiency; STAT, signal transducer and activator of transcription; XLA, X-linked agammaglobulinemia.