3A01.0

ICD-11 MMS 2026-01

Hereditary vitamin B12 deficiency anaemia

Description

This is a hereditary low blood level of vitamin B12. It can cause permanent damage to nervous tissue if left untreated long enough. Vitamin B12 itself was discovered through investigation of pernicious anaemia, which is an autoimmune disease that destroys parietal cells in the stomach that secrete intrinsic factor.

Index terms

  • Hereditary vitamin B12 deficiency anaemia
  • congenital intrinsic factor deficiency
  • defects of cobalamin metabolism
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  • megaloblastic anaemia due to hereditary vitamin B12 deficiency
  • Hereditary megaloblastic anaemia due to transcobalamin deficiency
  • Congenital megaloblastic anaemia due to transcobalamin II deficiency
  • TCN2 - [transcobalamin II deficiency]
  • transcobalamin II deficiency
  • transcobalamin II deficiency anaemia
  • transcobalamin deficiency
  • Anaemia due to selective hereditary vitamin B12 malabsorption with proteinuria

Exclusions from above levels

  • 04 – Diseases of the immune system
  • 05 – Endocrine, nutritional or metabolic diseases
  • 18 – Complications of pregnancy, childbirth or the puerperium
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  • 19 – Certain conditions originating in the perinatal period
  • 20 – Congenital malformations, deformations or chromosomal abnormalities
  • 22 – Injury, poisoning or certain other consequences of external causes
  • http://id.who.int/icd/entity/1000704511 – Human immunodeficiency virus disease
  • JB64.1 – Other diseases of the blood or blood-forming organs or certain disorders involving the immune mechanism complicating pregnancy, childbirth or the puerperium

Sibling codes

  • 3A01.1 – Neonatal vitamin B12 deficiency anaemia
  • 3A01.2 – Vitamin B12 deficiency anaemia due to low intake
  • 3A01.3 – Vitamin B12 deficiency anaemia due to intrinsic factor deficiency
  • 3A01.4 – Vitamin B12 deficiency anaemia due to intestinal disease
  • 3A01.5 – Drug-induced vitamin B12 deficiency anaemia
  • 3A01.Y – Other specified megaloblastic anaemia due to vitamin B12 deficiency
  • 3A01.Z – Megaloblastic anaemia due to vitamin B12 deficiency, unspecified

Foundation entity

Hereditary vitamin B12 deficiency anaemia – http://id.who.int/icd/entity/1463520192

Part of: 3A01: Megaloblastic anaemia due to vitamin B12 deficiency › 03: Diseases of the blood or blood-forming organs