4A01.11

ICD-11 MMS 2026-01

Major histocompatibility complex class I deficiency

Index terms

  • Major histocompatibility complex class I deficiency
  • BLS - [bare lymphocyte syndrome] NOS
  • Bare lymphocyte syndrome type 1
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  • Immunodeficiency by defective expression of HLA - [human leukocyte antigen] class 1
  • SCID - [severe combined immunodeficiency] due to absent class 2 HLA antigens

Exclusions from above levels

  • 4A01.00 – autosomal recessive agammaglobulinaemia (Swiss type)
  • 02 – Neoplasms
  • 18 – Complications of pregnancy, childbirth and the puerperium
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  • 20 – Developmental anomalies

Sibling codes

  • 4A01.10 – Severe combined immunodeficiencies
  • 4A01.12 – Major histocompatibility complex class II deficiency
  • 4A01.1Y – Other specified combined immunodeficiencies
  • 4A01.1Z – Combined immunodeficiencies, unspecified

Foundation entity

Major histocompatibility complex class I deficiency – http://id.who.int/icd/entity/489749747

Part of: 4A01.1: Combined immunodeficiencies › 4A01: Primary immunodeficiencies due to disorders of adaptive immunity › 04: Diseases of the immune system