Practice parameter for the diagnosis and management of primary immunodeficiency (Algorithm 3)

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3-13. Consider TAP1, TAP2, ZAP70, TAPBP 3-11. Consider MHC II defects 3-8. Consider IL7RA, complete DGS 3-5. Consider RAG1, RAG2, Artemis, ADA 3-9. Consider XSCID, JAK3, IL7RA 3-7. NK cells present? 3-4. B cells present? 3-14. Consider Omenn syndrome (RAG1, RAG2). May be undefined SCID or severe phenotype of the CID 3-12. Selective low CD8? 3-10. Selective low CD4? 3-6. T cells of maternal origin? 3-3. T cells present? 3-19. Diagnosis not established, may be undefined CID or atypical presentation of defined CID. Also consider severe presentation of humoral immunodeficiency. 3-18. Evaluate for specific molecular defect. 3-17. Characteristic features of defined CID syndromes? 3-16. Consider XLP, NEMO, WAS, other 3-15. NK function normal? 3-20. Go to Algorithm 2. 3-1. Suspected cellular or combined immunodeficiency: complete screening of humoral and cellular immune function. 3-2. Clinical or laboratory presentation consistent with SCID? Source: Joint Council of Allergy, Asthma and Immunology. Practice parameter for the diagnosis and management of primary immunodeficiency. J Allergy Clin Immunol 2005 May;94:S1-63. All copyrights are reserved by the Joint Council of Allergy, Asthma and Immunology. Practice parameter for the diagnosis and management of primary immunodeficiency (Algorithm 3)