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4A01.22 Immune dysregulation syndromes presenting primarily with lymphoproliferation

International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2024-01

synonyms

• Immune dysregulation syndromes presenting primarily with lymphoproliferation
• Autosomal recessive lymphoproliferative disease
• Autoimmune lymphoproliferative syndrome
• autoimmune lymphoproliferative syndrome with recurrent infections
• Canale-Smith syndrome
• FAS deficiency
• FAS deficiency (ALPS-FAS)(ALPS1a)
• Caspase 8 defect (ALPS2b)
• ALPS-NRAS
• ALPS-KRAS
• ALPS-FASLG (ALPS1b)
• ALPS-CASP10 (ALPS2a)
• X-linked lymphoproliferative disease
• Purtilo syndrome
• Immunodeficiency following hereditary defective response to Epstein-Barr virus
• immunodeficient hereditary defective response to Epstein Barr virus
• x-linked lymphoproliferation disease
• x-linked lymphoproliferative disease or disorder
• x-linked lymphoproliferative syndrome
• XLPS - [x-linked lymphoproliferative syndrome]
• Fatal Epstein Barr virus syndrome
• XIAP deficiency (XLP2)
• SH2D1A deficiency (XLP1)

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