5C64.10

ICD-11 MMS 2026-01

Iron overload diseases

Description

Iron overload is the accumulation of excess iron in body tissues. Iron overload usually occurs as a result of a genetic predisposition to absorb and store iron in excess amounts, the most common form of which is hereditary hemochromatosis. Iron overload can also occur as a complication of other hematologic disorders that require chronic transfusion therapy, repeated injections of parenteral iron, or excessive iron ingestion. Excessive iron stores usually accumulate in the reticuloendothelial tissues and cause little damage (“hemosiderosis”). If overload continues, iron eventually begins to accumulate in tissues such as hepatic parenchyma, pancreas, heart and synovium, causing hemochromatosis.

Index terms

  • Iron overload diseases
  • hemosiderosis
  • idiopathic haemosiderosis
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  • Hereditary haemochromatosis
  • HFE-related hereditary haemochromatosis, Type 1
  • C282Y/C282Y haemochromatosis
  • Classic haemochromatosis
  • Arthropathy in haemochromatosis
  • Juvenile hereditary haemochromatosis, Type 2
  • Juvenile hereditary haemochromatosis, Type 2A
  • Hemojuvelin-related haemochromatosis
  • Juvenile hereditary haemochromatosis, Type 2B
  • Hepcidin-related haemochromatosis
  • Non-HFE-related hereditary haemochromatosis, Type 3
  • TFR2-related haemochromatosis
  • Non-HFE-related hereditary haemochromatosis, Type 4
  • Autosomal dominant hereditary haemochromatosis
  • Ferroportin disease
  • Haemochromatosis due to defect in ferroportin
  • Secondary iron overload
  • African iron overload
  • Bantu haemosiderosis
  • Iron overload in sub-Saharan Africa
  • Dietary haemosiderosis
  • Neurodegeneration with brain iron accumulation
  • Hallervorden-Spatz disease (deprecated)
  • NBIA - [Neurodegeneration with brain iron accumulation]
  • pigmentary pallidal atrophy
  • pigmentary pallidal degeneration
  • progressive pigmentary pallidal degeneration
  • Mitochondrial Membrane Protein-Associated Neurodegeneration
  • MPAN - [Mitochondrial Membrane Protein-Associated Neurodegeneration]
  • NBIA4 - [Neurodegeneration with Brain Iron Accumulation type 4]
  • Neurodegeneration with Brain Iron Accumulation type 4
  • Neurodegeneration with brain iron accumulation due to C19orf12 mutation
  • Pantothenate-kinase-associated neurodegeneration
  • HARP - [hypoprebetalipoproteinemia, acanthocytosis, retinitis pigmentosa, and pallidal degeneration]
  • HSS - [Hallervorden-Spatz-Syndrome] (deprecated)
  • NBIA1 - [Neurodegeneration with brain iron accumulation type 1]
  • Neurodegeneration with brain iron accumulation type 1
  • PANK2 gene mutation-associated neurodegeneration
  • PKAN - [Pantothenate-kinase-associated neurodegeneration]
  • Atypical pantothenate kinase associated neurodegeneration
  • NBIA1 - [Neurodegeneration with brain iron accumulation type 1] atypical form
  • Neurodegeneration with brain iron accumulation type 1, atypical form
  • PKAN - [Pantothenate-kinase-associated neurodegeneration] atypical form
  • Classic pantothenate kinase associated neurodegeneration
  • NBIA1 - [Neurodegeneration with brain iron accumulation type 1] classic form
  • Neurodegeneration with brain iron accumulation type 1, classic form
  • PKAN - [Pantothenate-kinase-associated neurodegeneration] classic form
  • Beta-Propeller protein-associated neurodegeneration
  • BPAN - [Beta-Propeller protein-associated neurodegeneration]
  • NBIA5 - [Neurodegeneration with brain iron accumulation type 5]
  • neurodegeneration with brain iron accumulation type 5
  • COASY Protein-Associated Neurodegeneration
  • CoPAN - [COASY Protein-Associated Neurodegeneration]
  • PLA2G6-associated neurodegeneration
  • NBIA 2 PLA2G6 mutation
  • NBIA2 - [Neurodegeneration with brain iron accumulation type 2]
  • Neurodegeneration with brain iron accumulation type 2
  • PARK14 - [Parkinson disease 14]
  • PLAN - [PLA2G6-Associated Neurodegeneration]
  • Infantile neuroaxonal dystrophy
  • INAD - [Infantile neuroaxonal dystrophy]
  • Seitelberger disease (deprecated)
  • Kufor-Rakeb syndrome
  • KRS - [Kufor-Rakeb syndrome]
  • PARK9 - [Parkinson Disease Type 9]
  • Parkinson Disease Type 9
  • Autosomal recessive spastic paraplegia type 35
  • FAHN - [Fatty Acid Hydroxylase-Associated Neurodegeneration]
  • Fatty Acid Hydroxylase-Associated Neurodegeneration
  • SPG35 - [Spastic Paraplegia 35]
  • Spastic Paraplegia 35
  • Acquired hemochromatosis
  • Acquired iron overload
  • Neonatal haemochromatosis

Exclusions from above levels

  • 3A00 – Iron deficiency anaemia
  • 3A72 – Sideroblastic anaemia
  • 5B57 – Vitamin D deficiency
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  • 5B5K – dietary mineral deficiency
  • http://id.who.int/icd/entity/962023213 – Disorders of the parathyroids or parathyroid hormone system
  • 3A10.Z – Hereditary haemolytic anaemia due to enzyme deficiency
  • 5A71.01 – Congenital adrenal hyperplasia
  • 5A81.1 – 5-alpha-reductase deficiency
  • LD28.01 – Marfan syndrome
  • LD28.1 – Ehlers-Danlos syndrome
  • LD2A.4 – androgen resistance syndrome
  • 18 – Pregnancy, childbirth or the puerperium
  • http://id.who.int/icd/entity/1004987305 – Transitory endocrine or metabolic disorders specific to fetus or newborn

Foundation children elsewhere

Sibling codes

  • 5C64.1Y – Other specified disorders of iron metabolism
  • 5C64.1Z – Disorders of iron metabolism, unspecified

Foundation entity

Iron overload diseases – http://id.who.int/icd/entity/783417456

Part of: 5C64.1: Disorders of iron metabolism › 5C64: Disorders of mineral absorption or transport › 05: Endocrine, nutritional or metabolic diseases