4A01.10

ICD-11 MMS 2026-01

Severe combined immunodeficiencies

Description

Severe combined immunodeficiency (SCID) comprises a group of rare monogenic primary immunodeficiency disorders characterised by a lack of functional peripheral T lymphocytes resulting in early-onset severe respiratory infections and failure to thrive.

Index terms

  • Severe combined immunodeficiencies
  • SCID - [Severe combined immunodeficiencies]
  • severe combined immunodeficiency disease
Show allHide
  • Severe combined immunodeficiency with reticular dysgenesis
  • Congenital aleukocytosis
  • De Vaal disease
  • Reticular dysgenesis
  • SCID - [Severe combined immunodeficiencies] with reticular dysgenesis
  • Severe combined immunodeficiency with leukopenia
  • generalised haematopoietic hypoplasia
  • Severe combined immunodeficiency with low T- and B-cell numbers
  • SCID - [Severe combined immunodeficiencies] with low t- and b-cell numbers
  • T- B- severe combined immunodeficiency
  • Severe combined immunodeficiency T- B- due to RAG1 deficiency
  • SCID - [severe combined immunodeficiency] T- B- due to RAG1 - [recombination-activating gene 1] deficiency
  • Severe combined immunodeficiency T- B- due to recombination activating gene 1deficiency
  • Severe combined immunodeficiency T- B- due to RAG2 deficiency
  • SCID - [severe combined immunodeficiency] T- B- due to RAG2 - [recombination-activating gene 2] deficiency
  • Severe combined immunodeficiency T- B- due to recombination activating gene 2 deficiency
  • Severe combined immunodeficiency with hypereosinophilia
  • Omenn syndrome
  • SCID - [severe combined immunodeficiency] with hypereosinophilia
  • SCID - [Severe combined immunodeficiency] due to DCLRE1C- [DNA cross-link repair 1C] deficiency
  • Agammaglobulinaemia, alymphocytotic type
  • SCID - [Severe combined immunodeficiency] due to Artemis protein deficiency
  • SCID - [Severe combined immunodeficiency] due to DNA cross-link repair protein 1C deficiency
  • SCID - [Severe combined immunodeficiency], Athabaskan type
  • Severe combined immunodeficiency [SCID], alymphocytotic type
  • Severe combined immunodeficiency due to LCK deficiency
  • SCID - [severe combined immunodeficiency due to LCK - [lymphocyte-specific protein tyrosine kinase] deficiency
  • SCID - [Severe combined immunodeficiency] due to DNA-PKcs deficiency
  • SCID - [Severe combined immunodeficiency] due to DNA-PKcs - [DNA-dependent protein kinase, catalytic subunit] deficiency
  • Severe combined immunodeficiency T- B- due to adenosine deaminase deficiency
  • ADA - [adenosine aminohydrolase] deficiency
  • ADA - [adenosine deaminase] deficiency
  • Adenosine deaminase deficiency
  • SCID - [severe combined immunodeficiency] T- B- due to adenosine deaminase deficiency
  • Short-limb skeletal dysplasia with severe combined immunodeficiency
  • Achondroplasia - severe combined immunodeficiency
  • Achondroplasia or Swiss-type agammaglobulinaemia
  • Immunodeficiency with short limb dwarfism
  • SCID - [severe combined immunodeficiency] with short-limbed dwarfism
  • immunodeficiency with short-limbed stature
  • lymphopenic agammaglobulinaemia - short-limbed dwarfism syndrome
  • severe combined immunodeficiency with short-limbed dwarfism
  • short-limbed immunodeficiency
  • Severe combined immunodeficiency with low or normal B-cell numbers
  • SCID - [severe combined immunodeficiency] with low or normal b-cell numbers
  • T- B+ severe combined immunodeficiency
  • Severe combined immunodeficiency T- B+ due to gamma chain deficiency
  • SCID - [severe combined immunodeficiency] T- B+ due to gamma chain deficiency
  • Severe combined immunodeficiency T- B+, X-linked
  • Severe combined immunodeficiency T- B+ due to JAK3 deficiency
  • SCID - [severe combined immunodeficiency] T- B+ due to JAK3- [Janus kinase 3] deficiency
  • Severe combined immunodeficiency T- B+ due to IL-7Ralpha deficiency
  • SCID - [severe combined immunodeficiency] T- B+ due to IL-7Ralpha - [Interleukin 7 receptor alpha] deficiency
  • Severe combined immunodeficiency T-B+ due to CD45 deficiency
  • SCID - [severe combined immunodeficiency] T- B+ due to CD45 deficiency
  • Severe combined immunodeficiency T- B+ due to CD3delta, CD3epsilon or CD3zeta
  • SCID - [severe combined immunodeficiency] T- B+ due to CD3delta/CD3epsilon/CD3zeta
  • Severe combined immunodeficiency due to CORO1A deficiency
  • SCID - [severe combined immunodeficiency] due to coronin-1A deficiency

Exclusions from above levels

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

Sibling codes

  • 4A01.11 – Major histocompatibility complex class I deficiency
  • 4A01.12 – Major histocompatibility complex class II deficiency
  • 4A01.1Y – Other specified combined immunodeficiencies
  • 4A01.1Z – Combined immunodeficiencies, unspecified

Foundation entity

Severe combined immunodeficiencies – http://id.who.int/icd/entity/963193284

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