ICD-10-CM Book 2024 Edition

Chapters  /  Chapter 3  /  D55-D59

Hemolytic anemias (D55-D59)

D55 Anemia due to enzyme disorders
Excludes1 drug-induced enzyme deficiency anemia (D59.2)
D55.0 Anemia due to glucose-6-phosphate dehydrogenase [G6PD] deficiency
Favism
G6PD deficiency anemia
Excludes1 glucose-6-phosphate dehydrogenase (G6PD) deficiency without anemia (D75.A)
D55.1 Anemia due to other disorders of glutathione metabolism
Anemia (due to) enzyme deficiencies, except G6PD, related to the hexose monophosphate [HMP] shunt pathway
Anemia (due to) hemolytic nonspherocytic (hereditary), type I
D55.2 Anemia due to disorders of glycolytic enzymes
Excludes1 disorders of glycolysis not associated with anemia (E74.81-)
D55.21 Anemia due to pyruvate kinase deficiency
PK deficiency anemia
Pyruvate kinase deficiency anemia
D55.29 Anemia due to other disorders of glycolytic enzymes
Hexokinase deficiency anemia
Triose-phosphate isomerase deficiency anemia
D55.3 Anemia due to disorders of nucleotide metabolism
D55.8 Other anemias due to enzyme disorders
D55.9 Anemia due to enzyme disorder, unspecified
D56 Thalassemia
Excludes1 sickle-cell thalassemia (D57.4-)
D56.0 Alpha thalassemia
Alpha thalassemia major
Hemoglobin H Constant Spring
Hemoglobin H disease
Hydrops fetalis due to alpha thalassemia
Severe alpha thalassemia
Triple gene defect alpha thalassemia
UseAdditionalCode code, if applicable, for hydrops fetalis due to alpha thalassemia (P56.99)
Excludes1 alpha thalassemia trait or minor (D56.3)
asymptomatic alpha thalassemia (D56.3)
hydrops fetalis due to isoimmunization (P56.0)
hydrops fetalis not due to immune hemolysis (P83.2)
D56.1 Beta thalassemia
Beta thalassemia major
Cooley's anemia
Homozygous beta thalassemia
Severe beta thalassemia
Thalassemia intermedia
Thalassemia major
Excludes1 beta thalassemia minor (D56.3)
beta thalassemia trait (D56.3)
delta-beta thalassemia (D56.2)
hemoglobin E-beta thalassemia (D56.5)
sickle-cell beta thalassemia (D57.4-)
D56.2 Delta-beta thalassemia
Homozygous delta-beta thalassemia
Excludes1 delta-beta thalassemia minor (D56.3)
delta-beta thalassemia trait (D56.3)
D56.3 Thalassemia minor
Alpha thalassemia minor
Alpha thalassemia silent carrier
Alpha thalassemia trait
Beta thalassemia minor
Beta thalassemia trait
Delta-beta thalassemia minor
Delta-beta thalassemia trait
Thalassemia trait NOS
Excludes1 alpha thalassemia (D56.0)
beta thalassemia (D56.1)
delta-beta thalassemia (D56.2)
hemoglobin E-beta thalassemia (D56.5)
sickle-cell trait (D57.3)
D56.4 Hereditary persistence of fetal hemoglobin [HPFH]
D56.5 Hemoglobin E-beta thalassemia
Excludes1 beta thalassemia (D56.1)
beta thalassemia minor (D56.3)
beta thalassemia trait (D56.3)
delta-beta thalassemia (D56.2)
delta-beta thalassemia trait (D56.3)
hemoglobin E disease (D58.2)
other hemoglobinopathies (D58.2)
sickle-cell beta thalassemia (D57.4-)
D56.8 Other thalassemias
Dominant thalassemia
Hemoglobin C thalassemia
Mixed thalassemia
Thalassemia with other hemoglobinopathy
Excludes1 hemoglobin C disease (D58.2)
hemoglobin E disease (D58.2)
other hemoglobinopathies (D58.2)
sickle-cell anemia (D57.-)
sickle-cell thalassemia (D57.4-)
D56.9 Thalassemia, unspecified
Mediterranean anemia (with other hemoglobinopathy)
D57 Sickle-cell disorders
UseAdditionalCode code for any associated fever (R50.81)
Excludes1 other hemoglobinopathies (D58.-)
D57.0 Hb-SS disease with crisis
Sickle-cell disease with crisis
Hb-SS disease with (vaso-occlusive) pain
D57.00 Hb-SS disease with crisis, unspecified
Hb-SS disease with (painful) crisis NOS
Hb-SS disease with (vaso-occlusive) pain NOS
D57.01 Hb-SS disease with acute chest syndrome
D57.02 Hb-SS disease with splenic sequestration
D57.03 Hb-SS disease with cerebral vascular involvement
CodeAlso , if applicable, cerebral infarction (I63.-)
D57.04 Hb-SS disease with dactylitis
D57.09 Hb-SS disease with crisis with other specified complication
UseAdditionalCode code to identify complications, such as:
cholelithiasis (K80.-)
priapism (N48.32)
D57.1 Sickle-cell disease without crisis
Hb-SS disease without crisis
Sickle-cell anemia NOS
Sickle-cell disease NOS
Sickle-cell disorder NOS
D57.2 Sickle-cell/Hb-C disease
Hb-SC disease
Hb-S/Hb-C disease
D57.20 Sickle-cell/Hb-C disease without crisis
D57.21 Sickle-cell/Hb-C disease with crisis
D57.211 Sickle-cell/Hb-C disease with acute chest syndrome
D57.212 Sickle-cell/Hb-C disease with splenic sequestration
D57.213 Sickle-cell/Hb-C disease with cerebral vascular involvement
CodeAlso , if applicable, cerebral infarction (I63.-)
D57.214 Sickle-cell/Hb-C disease with dactylitis
D57.218 Sickle-cell/Hb-C disease with crisis with other specified complication
UseAdditionalCode code to identify complications, such as:
cholelithiasis (K80.-)
priapism (N48.32)
D57.219 Sickle-cell/Hb-C disease with crisis, unspecified
Sickle-cell/Hb-C disease with crisis NOS
Sickle-cell/Hb-C disease with (vaso-occlusive) pain NOS
D57.3 Sickle-cell trait
Hb-S trait
Heterozygous hemoglobin S
D57.4 Sickle-cell thalassemia
Sickle-cell beta thalassemia
Thalassemia Hb-S disease
D57.40 Sickle-cell thalassemia without crisis
Microdrepanocytosis
Sickle-cell thalassemia NOS
D57.41 Sickle-cell thalassemia, unspecified, with crisis
Sickle-cell thalassemia with (painful) crisis NOS
Sickle-cell thalassemia with (vaso-occlusive) pain NOS
D57.411 Sickle-cell thalassemia, unspecified, with acute chest syndrome
D57.412 Sickle-cell thalassemia, unspecified, with splenic sequestration
D57.413 Sickle-cell thalassemia, unspecified, with cerebral vascular involvement
CodeAlso , if applicable cerebral infarction (I63.-)
D57.414 Sickle-cell thalassemia, unspecified, with dactylitis
D57.418 Sickle-cell thalassemia, unspecified, with crisis with other specified complication
UseAdditionalCode code to identify complications, such as:
cholelithiasis (K80.-)
priapism (N48.32)
D57.419 Sickle-cell thalassemia, unspecified, with crisis
Sickle-cell thalassemia with (painful) crisis NOS
Sickle-cell thalassemia with (vaso-occlusive) pain NOS
D57.42 Sickle-cell thalassemia beta zero without crisis
HbS-beta zero without crisis
Sickle-cell beta zero without crisis
D57.43 Sickle-cell thalassemia beta zero with crisis
HbS-beta zero with crisis
Sickle-cell beta zero with crisis
D57.431 Sickle-cell thalassemia beta zero with acute chest syndrome
HbS-beta zero with acute chest syndrome
Sickle-cell beta zero with acute chest syndrome
D57.432 Sickle-cell thalassemia beta zero with splenic sequestration
HbS-beta zero with splenic sequestration
Sickle-cell beta zero with splenic sequestration
D57.433 Sickle-cell thalassemia beta zero with cerebral vascular involvement
HbS-beta zero with cerebral vascular involvement
Sickle-cell beta zero with cerebral vascular involvement
CodeAlso , if applicable cerebral infarction (I63.-)
D57.434 Sickle-cell thalassemia beta zero with dactylitis
D57.438 Sickle-cell thalassemia beta zero with crisis with other specified complication
HbS-beta zero with other specified complication
Sickle-cell beta zero with other specified complication
UseAdditionalCode code to identify complications, such as:
cholelithiasis (K80.-)
priapism (N48.32)
D57.439 Sickle-cell thalassemia beta zero with crisis, unspecified
HbS-beta zero with other specified complication
Sickle-cell beta zero with crisis unspecified
Sickle-cell thalassemia beta zero with (painful) crisis NOS
Sickle-cell thalassemia beta zero with (vaso-occlusive) pain NOS
D57.44 Sickle-cell thalassemia beta plus without crisis
HbS-beta plus without crisis
Sickle-cell beta plus without crisis
D57.45 Sickle-cell thalassemia beta plus with crisis
HbS-beta plus with crisis
Sickle-cell beta plus with crisis
D57.451 Sickle-cell thalassemia beta plus with acute chest syndrome
HbS-beta plus with acute chest syndrome
Sickle-cell beta plus with acute chest syndrome
D57.452 Sickle-cell thalassemia beta plus with splenic sequestration
HbS-beta plus with splenic sequestration
Sickle-cell beta plus with splenic sequestration
D57.453 Sickle-cell thalassemia beta plus with cerebral vascular involvement
HbS-beta plus with cerebral vascular involvement
Sickle-cell beta plus with cerebral vascular involvement
CodeAlso , if applicable cerebral infarction (I63.-)
D57.454 Sickle-cell thalassemia beta plus with dactylitis
D57.458 Sickle-cell thalassemia beta plus with crisis with other specified complication
HbS-beta plus with crisis with other specified complication
Sickle-cell beta plus with crisis with other specified complication
UseAdditionalCode code to identify complications, such as:
cholelithiasis (K80.-)
priapism (N48.32)
D57.459 Sickle-cell thalassemia beta plus with crisis, unspecified
HbS-beta plus with crisis with unspecified complication
Sickle-cell beta plus with crisis with unspecified complication
Sickle-cell thalassemia beta plus with (painful) crisis NOS
Sickle-cell thalassemia beta plus with (vaso-occlusive) pain NOS
D57.8 Other sickle-cell disorders
Hb-SD disease
Hb-SE disease
D57.80 Other sickle-cell disorders without crisis
D57.81 Other sickle-cell disorders with crisis
D57.811 Other sickle-cell disorders with acute chest syndrome
D57.812 Other sickle-cell disorders with splenic sequestration
D57.813 Other sickle-cell disorders with cerebral vascular involvement
CodeAlso , if applicable: cerebral infarction (I63.-)
D57.814 Other sickle-cell disorders with dactylitis
D57.818 Other sickle-cell disorders with crisis with other specified complication
UseAdditionalCode code to identify complications, such as:
cholelithiasis (K80.-)
priapism (N48.32)
D57.819 Other sickle-cell disorders with crisis, unspecified
Other sickle-cell disorders with crisis NOS
Other sickle-cell disorders with (vaso-occlusive) pain NOS
D58 Other hereditary hemolytic anemias
Excludes1 hemolytic anemia of the newborn (P55.-)
D58.0 Hereditary spherocytosis
Acholuric (familial) jaundice
Congenital (spherocytic) hemolytic icterus
Minkowski-Chauffard syndrome
D58.1 Hereditary elliptocytosis
Elliptocytosis (congenital)
Ovalocytosis (congenital) (hereditary)
D58.2 Other hemoglobinopathies
Abnormal hemoglobin NOS
Congenital Heinz body anemia
Hb-C disease
Hb-D disease
Hb-E disease
Hemoglobinopathy NOS
Unstable hemoglobin hemolytic disease
Excludes1 familial polycythemia (D75.0)
Hb-M disease (D74.0)
hemoglobin E-beta thalassemia (D56.5)
hereditary persistence of fetal hemoglobin [HPFH] (D56.4)
high-altitude polycythemia (D75.1)
methemoglobinemia (D74.-)
other hemoglobinopathies with thalassemia (D56.8)
D58.8 Other specified hereditary hemolytic anemias
Stomatocytosis
D58.9 Hereditary hemolytic anemia, unspecified
D59 Acquired hemolytic anemia
D59.0 Drug-induced autoimmune hemolytic anemia
UseAdditionalCode code for adverse effect, if applicable, to identify drug (T36-T50 with fifth or sixth character 5)
D59.1 Other autoimmune hemolytic anemias
Excludes2 Evans syndrome (D69.41)
hemolytic disease of newborn (P55.-)
paroxysmal cold hemoglobinuria (D59.6)
D59.10 Autoimmune hemolytic anemia, unspecified
D59.11 Warm autoimmune hemolytic anemia
Warm type (primary) (secondary) (symptomatic) autoimmune hemolytic anemia
Warm type autoimmune hemolytic disease
D59.12 Cold autoimmune hemolytic anemia
Chronic cold hemagglutinin disease
Cold agglutinin disease
Cold agglutinin hemoglobinuria
Cold type (primary) (secondary) (symptomatic) autoimmune hemolytic anemia
Cold type autoimmune hemolytic disease
D59.13 Mixed type autoimmune hemolytic anemia
Mixed type autoimmune hemolytic disease
Mixed type, cold and warm, (primary) (secondary) (symptomatic) autoimmune hemolytic anemia
D59.19 Other autoimmune hemolytic anemia
D59.2 Drug-induced nonautoimmune hemolytic anemia
Drug-induced enzyme deficiency anemia
UseAdditionalCode code for adverse effect, if applicable, to identify drug (T36-T50 with fifth or sixth character 5)
D59.3 Hemolytic-uremic syndrome
CodeAlso , if applicable, any associated:
acute kidney failure (N17.-)
chronic kidney disease (N18.-)
D59.30 Hemolytic-uremic syndrome, unspecified
Hemolytic-uremic syndrome NOS
D59.31 Infection-associated hemolytic-uremic syndrome
Shiga toxin-producing E. coli [STEC] related hemolytic uremic syndrome
Typical hemolytic uremic syndrome
UseAdditionalCode code to identify associated infection, such as :
E. coli infection (B96.2-)
Human immunodeficiency virus [HIV] disease (B20)
Pneumococcal meningitis (G00.1)
Pneumococcal pneumonia (J13)
Sepsis due to Streptococcus pneumoniae (A40.3)
Shigella dysenteriae (A03.9)
Streptococcus pneumoniae as the cause of diseases classified elsewhere (B95.3)
D59.32 Hereditary hemolytic-uremic syndrome
Atypical hemolytic uremic syndrome with an identified genetic cause
CodeAlso , if applicable:
defects in the complement system (D84.1)
methylmalonic acidemia (E71.120)
D59.39 Other hemolytic-uremic syndrome
Atypical (nongenetic) hemolytic uremic syndrome
Secondary hemolytic-uremic syndrome
CodeFirst , if applicable, any associated:
COVID-19 (U07.1)
complications of kidney transplant (T86.1-)
complications of heart transplant (T86.2-)
complications of liver transplant (T86.4-)
CodeAlso , if applicable, any associated condition, such as:
hypertensive emergency (I16.1)
malignant neoplasm (C00-C96)
systemic lupus erythematosus (M32.-)
UseAdditionalCode code, if applicable, for adverse effect to identify drug (T36-T50 with fifth or sixth character 5)
D59.4 Other nonautoimmune hemolytic anemias
Mechanical hemolytic anemia
Microangiopathic hemolytic anemia
Toxic hemolytic anemia
D59.5 Paroxysmal nocturnal hemoglobinuria [Marchiafava-Micheli]
Excludes1 hemoglobinuria NOS (R82.3)
D59.6 Hemoglobinuria due to hemolysis from other external causes
Hemoglobinuria from exertion
March hemoglobinuria
Paroxysmal cold hemoglobinuria
UseAdditionalCode code (Chapter 20) to identify external cause
Excludes1 hemoglobinuria NOS (R82.3)
D59.8 Other acquired hemolytic anemias
D59.9 Acquired hemolytic anemia, unspecified
Idiopathic hemolytic anemia, chronic