Chapter III

Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism

Haemolytic anaemias

D55 Anaemia due to enzyme disorders
Excludes: drug-induced enzyme deficiency anaemia ( D59.2 )
D55.0 Anaemia due to glucose-6-phosphate dehydrogenase [G6PD] deficiency
G6PD deficiency anaemia
D55.1 Anaemia due to other disorders of glutathione metabolism
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
· 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
Alpha thalassaemia
Haemoglobin H disease
Excludes: hydrops fetalis due to haemolytic disease ( P56.-)
D56.1 Beta thalassaemia
# Beta thalassaemia/haemoglobin E

Cooley's anaemia
Severe beta thalassaemia
Sickle-cell beta 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
Mediterranean anaemia (with other haemoglobinopathy)
Thalassaemia (minor)(mixed)(with other haemoglobinopathy)

D57 Sickle-cell disorders

Excludes: other haemoglobinopathies ( D58.- )
sickle-cell eta thalassaemia (D56.1)
D57.0 Sickle-cell anaemia with crisis
Hb-SS disease with crisis
D57.1 Sickle-cell anaemia without crisis
· anaemia
· disease
· disorder
D57.2 Double heterozygous sickling disorders
· Hb-SC
· Hb-SD
· Hb-SE
· sickle-cell thalassaemia
D57.3 Sickle-cell trait
Hb-S trait
Heterozygous haemoglobin S
D57.8 Other sickle-cell disorders

D58 Other hereditary haemolytic anaemias
D58.0 Hereditary spherocytosis
Acholuric (familial) jaundice
Congenital (spherocytic) haemolytic icterus
Minkowski-Chauffard syndrome
D58.1 Hereditary elliptocytosis
Elliptocytosis (congenital)
Ovalocytosis (congenital)(hereditary)
D58.2 Other haemoglobinopathies
Abnormal haemoglobin NOS
Congenital Heinz body anaemia
· Hb-C
· Hb-D
· Hb-E
Haemoglobinopathy NOS
Unstable haemoglobin haemolytic disease
Excludes: 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
D58.9 Hereditary haemolytic anaemia, unspecified

D59 Acquired haemolytic anaemia
D59.0 Drug-induced autoimmune haemolytic anaemia
Use additional external cause code (Chapter XX), if desired, to identify drug.
D59.1 Other autoimmune haemolytic anaemias
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)
Excludes: Evans' syndrome ( D69.3)
haemolytic disease of fetus and newborn ( P55.-)
paroxysmal cold haemoglobinuria ( D59.6)
D59.2 Drug-induced nonautoimmune haemolytic anaemia
Drug-induced enzyme deficiency anaemia
Use additional external cause code (Chapter XX), if desired, to identify drug.
D59.3 Haemolytic-uraemic syndrome
D59.4 Other nonautoimmune haemolytic anaemias

Use additional external cause code (Chapter XX), if desired, to identify cause.
#D59.40 Chemical
Excludes: Toxic
#D59.41 Infectious
#D59.42 Microangiopathic
#D59.43 Physical
Excludes: Mechanical
#D59.48 Other nonautoimmune haemolytic anaemias
D59.5 Paroxysmal nocturnal haemoglobinuria [Marchiafava-Micheli]
Excludes: haemoglobinuria NOS ( R82.3)
D59.6 Haemoglobinuria due to haemolysis from other external causes
· from exertion
· march
· paroxysmal cold
Use additional external cause code (Chapter XX), if desired, to identify cause.
Excludes: haemoglobinuria NOS ( R82.3)
D59.8 Other acquired haemolytic anaemias
D59.9 Acquired haemolytic anaemia, unspecified
Idiopathic haemolytic anaemia, chronic