Hemolytic anemias (D55-D59)
D55Anemia due to enzyme disorders
Excludes 1: drug-induced enzyme deficiency anemia (D59.2)
D55.0Anemia due to glucose-6-phosphate dehydrogenase [G6PD] deficiency
› Favism
› G6PD deficiency anemia
Excludes 1: glucose-6-phosphate dehydrogenase (G6PD) deficiency without anemia (D75.A)
D55.1Anemia 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
D55.2Anemia due to disorders of glycolytic enzymes
Excludes 1: disorders of glycolysis not associated with anemia (E74.81-)
D55.21Anemia due to pyruvate kinase deficiency
› PK deficiency anemia
› Pyruvate kinase deficiency anemia
D55.29Anemia due to other disorders of glycolytic enzymes
› Hexokinase deficiency anemia
› Triose-phosphate isomerase deficiency anemia
D55.3Anemia due to disorders of nucleotide metabolism
D55.8Other anemias due to enzyme disorders
D55.9Anemia due to enzyme disorder, unspecified
D56Thalassemia
Excludes 1: sickle-cell thalassemia (D57.4-)
D56.0Alpha 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
Use Additional Code: code, if applicable, for hydrops fetalis due to alpha thalassemia (P56.99)
Excludes 1: 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)
D56.1Beta thalassemia
› Beta thalassemia major
› Cooley's anemia
› Homozygous beta thalassemia
› Severe beta thalassemia
› Thalassemia intermedia
› Thalassemia major
Excludes 1: 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-)
D56.2Delta-beta thalassemia
› Homozygous delta-beta thalassemia
Excludes 1: delta-beta thalassemia minor (D56.3)
› delta-beta thalassemia trait (D56.3)
D56.3Thalassemia 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
Excludes 1: alpha thalassemia (D56.0)
› beta thalassemia (D56.1)
› delta-beta thalassemia (D56.2)
› hemoglobin E-beta thalassemia (D56.5)
› sickle-cell trait (D57.3)
D56.4Hereditary persistence of fetal hemoglobin [HPFH]
D56.5Hemoglobin E-beta thalassemia
Excludes 1: 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-)
D56.8Other thalassemias
› Dominant thalassemia
› Hemoglobin C thalassemia
› Mixed thalassemia
› Thalassemia with other hemoglobinopathy
Excludes 1: hemoglobin C disease (D58.2)
› hemoglobin E disease (D58.2)
› other hemoglobinopathies (D58.2)
› sickle-cell anemia (D57.-)
› sickle-cell thalassemia (D57.4-)
D56.9Thalassemia, unspecified
› Mediterranean anemia (with other hemoglobinopathy)
D57Sickle-cell disorders
Use Additional Code: code for any associated fever (R50.81)
Excludes 1: other hemoglobinopathies (D58.-)
D57.0Hb-SS disease with crisis
› Sickle-cell disease with crisis
› Hb-SS disease with (vaso-occlusive) pain
D57.00Hb-SS disease with crisis, unspecified
› Hb-SS disease with (painful) crisis NOS
› Hb-SS disease with (vaso-occlusive) pain NOS
D57.01Hb-SS disease with acute chest syndrome
D57.02Hb-SS disease with splenic sequestration
D57.03Hb-SS disease with cerebral vascular involvement
Code Also: , if applicable, cerebral infarction (I63.-)
D57.04Hb-SS disease with dactylitis
D57.09Hb-SS disease with crisis with other specified complication
Use Additional Code: code to identify complications, such as:
› cholelithiasis (K80.-)
› priapism (N48.32)
D57.1Sickle-cell disease without crisis
› Hb-SS disease without crisis
› Sickle-cell anemia NOS
› Sickle-cell disease NOS
› Sickle-cell disorder NOS
D57.2Sickle-cell/Hb-C disease
› Hb-SC disease
› Hb-S/Hb-C disease
D57.20Sickle-cell/Hb-C disease without crisis
D57.21Sickle-cell/Hb-C disease with crisis
D57.211Sickle-cell/Hb-C disease with acute chest syndrome
D57.212Sickle-cell/Hb-C disease with splenic sequestration
D57.213Sickle-cell/Hb-C disease with cerebral vascular involvement
Code Also: , if applicable, cerebral infarction (I63.-)
D57.214Sickle-cell/Hb-C disease with dactylitis
D57.218Sickle-cell/Hb-C disease with crisis with other specified complication
Use Additional Code: code to identify complications, such as:
› cholelithiasis (K80.-)
› priapism (N48.32)
D57.219Sickle-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
D57.3Sickle-cell trait
› Hb-S trait
› Heterozygous hemoglobin S
D57.4Sickle-cell thalassemia
› Sickle-cell beta thalassemia
› Thalassemia Hb-S disease
D57.40Sickle-cell thalassemia without crisis
› Microdrepanocytosis
› Sickle-cell thalassemia NOS
D57.41Sickle-cell thalassemia, unspecified, with crisis
› Sickle-cell thalassemia with (painful) crisis NOS
› Sickle-cell thalassemia with (vaso-occlusive) pain NOS
D57.411Sickle-cell thalassemia, unspecified, with acute chest syndrome
D57.412Sickle-cell thalassemia, unspecified, with splenic sequestration
D57.413Sickle-cell thalassemia, unspecified, with cerebral vascular involvement
Code Also: , if applicable cerebral infarction (I63.-)
D57.414Sickle-cell thalassemia, unspecified, with dactylitis
D57.418Sickle-cell thalassemia, unspecified, with crisis with other specified complication
Use Additional Code: code to identify complications, such as:
› cholelithiasis (K80.-)
› priapism (N48.32)
D57.419Sickle-cell thalassemia, unspecified, with crisis
› Sickle-cell thalassemia with (painful) crisis NOS
› Sickle-cell thalassemia with (vaso-occlusive) pain NOS
D57.42Sickle-cell thalassemia beta zero without crisis
› HbS-beta zero without crisis
› Sickle-cell beta zero without crisis
D57.43Sickle-cell thalassemia beta zero with crisis
› HbS-beta zero with crisis
› Sickle-cell beta zero with crisis
D57.431Sickle-cell thalassemia beta zero with acute chest syndrome
› HbS-beta zero with acute chest syndrome
› Sickle-cell beta zero with acute chest syndrome
D57.432Sickle-cell thalassemia beta zero with splenic sequestration
› HbS-beta zero with splenic sequestration
› Sickle-cell beta zero with splenic sequestration
D57.433Sickle-cell thalassemia beta zero with cerebral vascular involvement
› HbS-beta zero with cerebral vascular involvement
› Sickle-cell beta zero with cerebral vascular involvement
Code Also: , if applicable cerebral infarction (I63.-)
D57.434Sickle-cell thalassemia beta zero with dactylitis
D57.438Sickle-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
Use Additional Code: code to identify complications, such as:
› cholelithiasis (K80.-)
› priapism (N48.32)
D57.439Sickle-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
D57.44Sickle-cell thalassemia beta plus without crisis
› HbS-beta plus without crisis
› Sickle-cell beta plus without crisis
D57.45Sickle-cell thalassemia beta plus with crisis
› HbS-beta plus with crisis
› Sickle-cell beta plus with crisis
D57.451Sickle-cell thalassemia beta plus with acute chest syndrome
› HbS-beta plus with acute chest syndrome
› Sickle-cell beta plus with acute chest syndrome
D57.452Sickle-cell thalassemia beta plus with splenic sequestration
› HbS-beta plus with splenic sequestration
› Sickle-cell beta plus with splenic sequestration
D57.453Sickle-cell thalassemia beta plus with cerebral vascular involvement
› HbS-beta plus with cerebral vascular involvement
› Sickle-cell beta plus with cerebral vascular involvement
Code Also: , if applicable cerebral infarction (I63.-)
D57.454Sickle-cell thalassemia beta plus with dactylitis
D57.458Sickle-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
Use Additional Code: code to identify complications, such as:
› cholelithiasis (K80.-)
› priapism (N48.32)
D57.459Sickle-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
D57.8Other sickle-cell disorders
› Hb-SD disease
› Hb-SE disease
D57.80Other sickle-cell disorders without crisis
D57.81Other sickle-cell disorders with crisis
D57.811Other sickle-cell disorders with acute chest syndrome
D57.812Other sickle-cell disorders with splenic sequestration
D57.813Other sickle-cell disorders with cerebral vascular involvement
Code Also: , if applicable: cerebral infarction (I63.-)
D57.814Other sickle-cell disorders with dactylitis
D57.818Other sickle-cell disorders with crisis with other specified complication
Use Additional Code: code to identify complications, such as:
› cholelithiasis (K80.-)
› priapism (N48.32)
D57.819Other sickle-cell disorders with crisis, unspecified
› Other sickle-cell disorders with crisis NOS
› Other sickle-cell disorders with (vaso-occlusive) pain NOS
D58Other hereditary hemolytic anemias
Excludes 1: hemolytic anemia of the newborn (P55.-)
D58.0Hereditary spherocytosis
› Acholuric (familial) jaundice
› Congenital (spherocytic) hemolytic icterus
› Minkowski-Chauffard syndrome
D58.1Hereditary elliptocytosis
› Elliptocytosis (congenital)
› Ovalocytosis (congenital) (hereditary)
D58.2Other hemoglobinopathies
› Abnormal hemoglobin NOS
› Congenital Heinz body anemia
› Hb-C disease
› Hb-D disease
› Hb-E disease
› Hemoglobinopathy NOS
› Unstable hemoglobin hemolytic disease
Excludes 1: 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)
D58.8Other specified hereditary hemolytic anemias
› Stomatocytosis
D58.9Hereditary hemolytic anemia, unspecified
D59Acquired hemolytic anemia
D59.0Drug-induced autoimmune hemolytic anemia
Use Additional Code: code for adverse effect, if applicable, to identify drug (T36-T50) with fifth or sixth character 5)
D59.1Other autoimmune hemolytic anemias
Excludes 2: Evans syndrome (D69.41)
› hemolytic disease of newborn (P55.-)
› paroxysmal cold hemoglobinuria (D59.6)
D59.10Autoimmune hemolytic anemia, unspecified
D59.11Warm autoimmune hemolytic anemia
› Warm type (primary) (secondary) (symptomatic) autoimmune hemolytic anemia
› Warm type autoimmune hemolytic disease
D59.12Cold 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
D59.13Mixed type autoimmune hemolytic anemia
› Mixed type autoimmune hemolytic disease
› Mixed type, cold and warm, (primary) (secondary) (symptomatic) autoimmune hemolytic anemia
D59.19Other autoimmune hemolytic anemia
D59.2Drug-induced nonautoimmune hemolytic anemia
› Drug-induced enzyme deficiency anemia
Use Additional Code: code for adverse effect, if applicable, to identify drug (T36-T50) with fifth or sixth character 5)
D59.3Hemolytic-uremic syndrome
Code Also: , if applicable, any associated:
› acute kidney failure (N17.-)
› chronic kidney disease (N18.-)
D59.30Hemolytic-uremic syndrome, unspecified
› Hemolytic-uremic syndrome NOS
D59.31Infection-associated hemolytic-uremic syndrome
› Shiga toxin-producing E. coli [STEC] related hemolytic uremic syndrome
› Typical hemolytic uremic syndrome
Use Additional Code: 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)
D59.32Hereditary hemolytic-uremic syndrome
› Atypical hemolytic uremic syndrome with an identified genetic cause
Code Also: , if applicable:
› defects in the complement system (D84.1)
› methylmalonic acidemia (E71.120)
D59.39Other hemolytic-uremic syndrome
› Atypical (nongenetic) hemolytic uremic syndrome
› Secondary hemolytic-uremic syndrome
Code First: , 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-)
Code Also: , if applicable, any associated condition, such as:
› hypertensive emergency (I16.1)
› malignant neoplasm (C00-C96)
› systemic lupus erythematosus (M32.-)
Use Additional Code: code, if applicable, for adverse effect to identify drug (T36-T50 with fifth or sixth character 5)
D59.4Other nonautoimmune hemolytic anemias
› Mechanical hemolytic anemia
› Microangiopathic hemolytic anemia
› Toxic hemolytic anemia
D59.5Paroxysmal nocturnal hemoglobinuria [Marchiafava-Micheli]
Excludes 1: hemoglobinuria NOS (R82.3)
D59.6Hemoglobinuria due to hemolysis from other external causes
› Hemoglobinuria from exertion
› March hemoglobinuria
› Paroxysmal cold hemoglobinuria
Use Additional Code: code (Chapter 20) to identify external cause
Excludes 1: hemoglobinuria NOS (R82.3)
D59.8Other acquired hemolytic anemias
D59.9Acquired hemolytic anemia, unspecified
› Idiopathic hemolytic anemia, chronic