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  3. D50–D89
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  5. Sickle-cell disorders (D57)

Sickle-cell disorders (D57)

ICD-10 code section D57 is used to classify various sickle-cell disorders, which are genetic conditions affecting hemoglobin in red blood cells. These codes help specify whether a patient has sickle-cell disease, trait, or related complications, such as vaso-occlusive crises, acute chest syndrome, or splenic sequestration.

This section includes codes for sickle-cell anemia with crisis (like D57.0 for Hb-SS disease with crises including vaso-occlusive pain episodes), sickle-cell disease without crisis (D57.1), and variants like sickle-cell/Hb-C disease (D57.2) and sickle-cell thalassemia (D57.4). Synonyms such as "Sickle cell-hemoglobin SS disease," "Hand-foot syndrome," and "Mixed hemoglobin disorder" correspond to specific codes and help in identifying the precise diagnosis. Codes also distinguish between crises involving complications like acute chest syndrome and cerebral vascular involvement. The classification aids healthcare providers and coders in accurately documenting and managing sickle-cell conditions, ensuring the right clinical focus for treatment and billing. Using the correct ICD-10 code for sickle-cell disorder ensures proper identification of the disease type and associated complications.

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

    • Hemolytic anemias (D55-D59)

        • Sickle-cell disorders (D57)

        • D57 Sickle-cell disorders
        • D57.0 Hb-SS disease with crisis
        • D57.00 Hb-SS disease with crisis, unspecified
        • 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
        • D57.04 Hb-SS disease with dactylitis
        • D57.09 Hb-SS disease with crisis with other specified complication
        • D57.1 Sickle-cell disease without crisis
        • D57.2 Sickle-cell/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
        • D57.214 Sickle-cell/Hb-C disease with dactylitis
        • D57.218 Sickle-cell/Hb-C disease with crisis with other specified complication
        • D57.219 Sickle-cell/Hb-C disease with crisis, unspecified
        • D57.3 Sickle-cell trait
        • D57.4 Sickle-cell thalassemia
        • D57.40 Sickle-cell thalassemia without crisis
        • D57.41 Sickle-cell thalassemia, unspecified, with crisis
        • 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
        • D57.414 Sickle-cell thalassemia, unspecified, with dactylitis
        • D57.418 Sickle-cell thalassemia, unspecified, with crisis with other specified complication
        • D57.419 Sickle-cell thalassemia, unspecified, with crisis
        • D57.42 Sickle-cell thalassemia beta zero without crisis
        • D57.43 Sickle-cell thalassemia beta zero with crisis
        • D57.431 Sickle-cell thalassemia beta zero with acute chest syndrome
        • D57.432 Sickle-cell thalassemia beta zero with splenic sequestration
        • D57.433 Sickle-cell thalassemia beta zero with cerebral vascular involvement
        • D57.434 Sickle-cell thalassemia beta zero with dactylitis
        • D57.438 Sickle-cell thalassemia beta zero with crisis with other specified complication
        • D57.439 Sickle-cell thalassemia beta zero with crisis, unspecified
        • D57.44 Sickle-cell thalassemia beta plus without crisis
        • D57.45 Sickle-cell thalassemia beta plus with crisis
        • D57.451 Sickle-cell thalassemia beta plus with acute chest syndrome
        • D57.452 Sickle-cell thalassemia beta plus with splenic sequestration
        • D57.453 Sickle-cell thalassemia beta plus with cerebral vascular involvement
        • D57.454 Sickle-cell thalassemia beta plus with dactylitis
        • D57.458 Sickle-cell thalassemia beta plus with crisis with other specified complication
        • D57.459 Sickle-cell thalassemia beta plus with crisis, unspecified
        • D57.8 Other sickle-cell disorders
        • 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
        • D57.814 Other sickle-cell disorders with dactylitis
        • D57.818 Other sickle-cell disorders with crisis with other specified complication
        • D57.819 Other sickle-cell disorders with crisis, unspecified

Instructional Notations

Use Additional Code

The “use additional code” indicates that a secondary code could be used to further specify the patient’s condition. This note is not mandatory and is only used if enough information is available to assign an additional code.

  • code for any associated fever R50.81

Type 1 Excludes

A type 1 excludes note is a pure excludes note. It means "NOT CODED HERE!" An Excludes1 note indicates that the code excluded should never be used at the same time as the code above the Excludes1 note. An Excludes1 is used when two conditions cannot occur together, such as a congenital form versus an acquired form of the same condition.

  • other hemoglobinopathies D58

Clinical Terms

The following clinical terms provide additional context, helping users better understand the clinical background and common associations for each diagnosis listed in this section. Including related terms alongside ICD-10-CM codes supports coders, billers, and healthcare professionals in improving accuracy, enhancing documentation, and facilitating research or patient education.

Acute Chest Syndrome

Respiratory syndrome characterized by the appearance of a new pulmonary infiltrate on chest x-ray, accompanied by symptoms of fever, cough, chest pain, tachypnea, or DYSPNEA, often seen in patients with SICKLE CELL ANEMIA. Multiple factors (e.g., infection, and pulmonary FAT EMBOLISM) may contribute to the development of the syndrome.

Priapism

A prolonged painful erection that may lasts hours and is not associated with sexual activity. It is seen in patients with SICKLE CELL ANEMIA, advanced malignancy, spinal trauma; and certain drug treatments.

Sickle Cell Trait

The condition of being heterozygous for hemoglobin S.