4A01.22

ICD-11 MMS 2026-01

Immune dysregulation syndromes presenting primarily with lymphoproliferation

Index terms

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

Exclusions from above levels

  • 02 – Neoplasms
  • 18 – Complications of pregnancy, childbirth and the puerperium
  • 20 – Developmental anomalies

Sibling codes

  • 4A01.20 – Immune dysregulation syndromes with hypopigmentation
  • 4A01.21 – Immune dysregulation syndromes presenting primarily with autoimmunity
  • 4A01.23 – Primary haemophagocytic lymphohistiocytosis
  • 4A01.2Y – Other specified diseases of immune dysregulation
  • 4A01.2Z – Diseases of immune dysregulation, unspecified

Foundation entity

Immune dysregulation syndromes presenting primarily with lymphoproliferation – http://id.who.int/icd/entity/969875874

Part of: 4A01.2: Diseases of immune dysregulation › 4A01: Primary immunodeficiencies due to disorders of adaptive immunity › 04: Diseases of the immune system