Chapter III
Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism
(D50-D89)

Rules Conventions GCS

Haemolytic anaemias
(D55-D59)

D55 Anaemia due to enzyme disorders
DCS.XV.32

Excl.:
drug-induced enzyme deficiency anaemia ( D59.2)
D55.0 Anaemia due to glucose-6-phosphate dehydrogenase [G6PD] deficiency
Incl.:
Favism
G6PD deficiency anaemia
D55.1 Anaemia due to other disorders of glutathione metabolism
Incl.:
Anaemia (due to):
  • enzyme deficiencies, except G6PD, related to the hexose monophosphate [HMP] shunt pathway
  • haemolytic nonspherocytic (hereditary), type I
D55.2 Anaemia due to disorders of glycolytic enzymes
Incl.:
Anaemia:
  • haemolytic nonspherocytic (hereditary), type II
  • hexokinase deficiency
  • pyruvate kinase [PK] deficiency
  • triose-phosphate isomerase deficiency
D55.3 Anaemia due to disorders of nucleotide metabolism
D55.8 Other anaemias due to enzyme disorders
D55.9 Anaemia due to enzyme disorder, unspecified

D56 Thalassaemia
DCS.III.1 DCS.XV.32

D56.0 Alpha thalassaemia
Excl.:
hydrops fetalis due to haemolytic disease ( P56.-)
D56.1 Beta thalassaemia
Incl.:
Cooley anaemia
Severe beta thalassaemia
Thalassaemia:
  • intermedia
  • major
D56.2 Delta-beta thalassaemia
D56.3 Thalassaemia trait
D56.4 Hereditary persistence of fetal haemoglobin [HPFH]
D56.8 Other thalassaemias
D56.9 Thalassaemia, unspecified
Incl.:
Mediterranean anaemia (with other haemoglobinopathy)
Thalassaemia (minor)(mixed)(with other haemoglobinopathy)

D57 Sickle-cell disorders
DCS.XV.32

Excl.:
other haemoglobinopathies ( D58.-)
D57.0 Sickle-cell anaemia with crisis
DCS.III.1
Incl.:
Hb-SS disease with crisis
D57.1 Sickle-cell anaemia without crisis
DCS.III.1
Incl.:
  • Sickle-cell:
    • anaemia
    • disease
    • disorder
  • NOS
D57.2 Double heterozygous sickling disorders
Incl.:
Disease:
  • Hb-SC
  • Hb-SD
  • Hb-SE
  • sickle-cell thalassaemia
D57.3 Sickle-cell trait
DCS.III.1
Incl.:
Hb-S trait
Heterozygous haemoglobin S [HbAS]
D57.8 Other sickle-cell disorders

D58 Other hereditary haemolytic anaemias
DCS.XV.32

D58.0 Hereditary spherocytosis
Incl.:
Acholuric (familial) jaundice
Congenital (spherocytic) haemolytic icterus
Minkowski-Chauffard syndrome
D58.1 Hereditary elliptocytosis
Incl.:
Elliptocytosis (congenital)
Ovalocytosis (congenital)(hereditary)
D58.2 Other haemoglobinopathies
Incl.:
Abnormal haemoglobin NOS
Congenital Heinz body anaemia
Disease:
  • Hb-C
  • Hb-D
  • Hb-E
Haemoglobinopathy NOS
Unstable haemoglobin haemolytic disease
Excl.:
familial polycythaemia ( D75.0)
Hb-M disease ( D74.0)
hereditary persistence of fetal haemoglobin [HPFH] ( D56.4)
high-altitude polycythaemia ( D75.1)
methaemoglobinaemia ( D74.-)
D58.8 Other specified hereditary haemolytic anaemias
Incl.:
Stomatocytosis
D58.9 Hereditary haemolytic anaemia, unspecified

D59 Acquired haemolytic anaemia
DCS.XV.32

D59.0 Drug-induced autoimmune haemolytic anaemia
Coding-Hint
Use additional external cause code (Chapter XX), if desired, to identify drug.
D59.1 Other autoimmune haemolytic anaemias
Incl.:
Autoimmune haemolytic disease (cold type)(warm type)
Chronic cold haemagglutinin disease
Cold agglutinin:
  • disease
  • haemoglobinuria
Haemolytic anaemia:
  • cold type (secondary)(symptomatic)
  • warm type (secondary)(symptomatic)
Excl.:
Evans syndrome ( D69.3)
haemolytic disease of fetus and newborn ( P55.-)
paroxysmal cold haemoglobinuria ( D59.6)
D59.2 Drug-induced nonautoimmune haemolytic anaemia
Incl.:
Drug-induced enzyme deficiency anaemia
Coding-Hint
Use additional external cause code (Chapter XX), if desired, to identify drug.
D59.3 Haemolytic-uraemic syndrome
D59.4 Other nonautoimmune haemolytic anaemias
Incl.:
Haemolytic anaemia:
  • mechanical
  • microangiopathic
  • toxic
Coding-Hint
Use additional external cause code (Chapter XX), if desired, to identify cause.
D59.5 Paroxysmal nocturnal haemoglobinuria [Marchiafava-Micheli]
Excl.:
haemoglobinuria NOS ( R82.3)
D59.6 Haemoglobinuria due to haemolysis from other external causes
Incl.:
Haemoglobinuria:
  • from exertion
  • march
  • paroxysmal cold
Coding-Hint
Use additional external cause code (Chapter XX), if desired, to identify cause.
Excl.:
haemoglobinuria NOS ( R82.3)
D59.8 Other acquired haemolytic anaemias
D59.9 Acquired haemolytic anaemia, unspecified
Incl.:
Idiopathic haemolytic anaemia, chronic