4A01.31

ICD-11 MMS 2026-01

DNA repair defects other than combined T-cell or B-cell immunodeficiencies

Index terms

  • DNA repair defects other than combined T-cell or B-cell immunodeficiencies
  • Nijmegen breakage syndrome
  • AT V1- [Ataxia-telangiectasia, variant 1]
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  • Ataxia-telangiectasia, variant 1
  • Autosomal recessive nonsyndromal microcephaly with normal intelligence
  • Berlin breakage syndrome
  • Immunodeficiency - microcephaly - chromosomal instability
  • Microcephaly - immunodeficiency - lymphoreticuloma
  • NBS - [Nijmegen breakage syndrome]
  • Seemanova syndrome type 2
  • Nijmegen breakage syndrome-like disorder
  • Microcephaly or chromosomal instability without immunodeficiency
  • NBS-like disorder
  • NBSLD - [Nijmegen breakage syndrome-like disorder]
  • Bloom syndrome
  • Bloom Machacek syndrome
  • Bloom-Torre-Machacek syndrome
  • Ataxia-telangiectasia
  • Louis-Bar syndrome
  • Ataxia-telangiectasia-like disorder
  • ATLD - [Ataxia-telangiectasia-like disorder]

Exclusions from above levels

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

Sibling codes

  • 4A01.30 – Immunodeficiency due to defects of the thymus
  • 4A01.32 – Immuno-osseous dysplasia
  • 4A01.33 – Hepatic veno-occlusive disease - immunodeficiency syndrome
  • 4A01.34 – Hyperimmunoglobulin E syndromes

Foundation entity

DNA repair defects other than combined T-cell or B-cell immunodeficiencies – http://id.who.int/icd/entity/1362501774

Part of: 4A01.3: Other well-defined immunodeficiency syndromes due to defects in adaptive immunity › 4A01: Primary immunodeficiencies due to disorders of adaptive immunity › 04: Diseases of the immune system