1. D57 Sickle-cell disorders

    Use Additional code for any associated fever (R50.81)

    Excludes1

    • other hemoglobinopathies (D58.-)
    1. D57.0 Hb-SS disease with crisis

      Inclusion term(s):

      • Sickle-cell disease with crisis
      • Hb-SS disease with vasoocclusive pain
      1. D57.00 Hb-SS disease with crisis, unspecified

        Inclusion term(s):

        • Hb-SS disease with (painful) crisis NOS
        • Hb-SS disease with vasoocclusive pain NOS
      2. D57.01 Hb-SS disease with acute chest syndrome

      3. D57.02 Hb-SS disease with splenic sequestration

      4. D57.03 Hb-SS disease with cerebral vascular involvement

      5. D57.09 Hb-SS disease with crisis with other specified complication

    2. D57.1 Sickle-cell disease without crisis

      Inclusion term(s):

      • Hb-SS disease without crisis
      • Sickle-cell anemia NOS
      • Sickle-cell disease NOS
      • Sickle-cell disorder NOS
    3. D57.2 Sickle-cell/Hb-C disease

      Inclusion term(s):

      • Hb-SC disease
      • Hb-S/Hb-C disease
      1. D57.20 Sickle-cell/Hb-C disease without crisis

      2. 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
        • D57.218 Sickle-cell/Hb-C disease with crisis with other specified complication
        • D57.219 Sickle-cell/Hb-C disease with crisis, unspecified

          Inclusion term(s):

          • Sickle-cell/Hb-C disease with crisis NOS
          • Sickle-cell/Hb-C disease with vasoocclusive pain NOS
    4. D57.3 Sickle-cell trait

      Inclusion term(s):

      • Hb-S trait
      • Heterozygous hemoglobin S
    5. D57.4 Sickle-cell thalassemia

      Inclusion term(s):

      • Sickle-cell beta thalassemia
      • Thalassemia Hb-S disease
      1. D57.40 Sickle-cell thalassemia without crisis

        Inclusion term(s):

        • Microdrepanocytosis
        • Sickle-cell thalassemia NOS
      2. D57.41 Sickle-cell thalassemia, unspecified, with crisis

        Inclusion term(s):

        • Sickle-cell thalassemia with (painful) crisis NOS
        • Sickle-cell thalassemia with vasoocclusive 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
        • D57.418 Sickle-cell thalassemia, unspecified, with crisis with other specified complication
        • D57.419 Sickle-cell thalassemia, unspecified, with crisis

          Inclusion term(s):

          • Sickle-cell thalassemia with (painful) crisis NOS
          • Sickle-cell thalassemia with vasoocclusive pain NOS
      3. D57.42 Sickle-cell thalassemia beta zero without crisis

        Inclusion term(s):

        • HbS-beta zero without crisis
        • Sickle-cell beta zero without crisis
      4. D57.43 Sickle-cell thalassemia beta zero with crisis

        Inclusion term(s):

        • HbS-beta zero with crisis
        • Sickle-cell beta zero with crisis
        • D57.431 Sickle-cell thalassemia beta zero with acute chest syndrome

          Inclusion term(s):

          • 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

          Inclusion term(s):

          • HbS-beta zero with splenic sequestration
          • Sickle-cell beta zero with splenic sequestration
        • D57.433 Sickle-cell thalassemia beta zero with cerebral vascular involvement

          Inclusion term(s):

          • HbS-beta zero with cerebral vascular involvement
          • Sickle-cell beta zero with cerebral vascular involvement
        • D57.438 Sickle-cell thalassemia beta zero with crisis with other specified complication

          Inclusion term(s):

          • HbS-beta zero with other specified complication
          • Sickle-cell beta zero with other specified complication
        • D57.439 Sickle-cell thalassemia beta zero with crisis, unspecified

          Inclusion term(s):

          • 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 vasoocclusive pain NOS
      5. D57.44 Sickle-cell thalassemia beta plus without crisis

        Inclusion term(s):

        • HbS-beta plus without crisis
        • Sickle-cell beta plus without crisis
      6. D57.45 Sickle-cell thalassemia beta plus with crisis

        Inclusion term(s):

        • HbS-beta plus with crisis
        • Sickle-cell beta plus with crisis
        • D57.451 Sickle-cell thalassemia beta plus with acute chest syndrome

          Inclusion term(s):

          • 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

          Inclusion term(s):

          • HbS-beta plus with splenic sequestration
          • Sickle-cell beta plus with splenic sequestration
        • D57.453 Sickle-cell thalassemia beta plus with cerebral vascular involvement

          Inclusion term(s):

          • HbS-beta plus with cerebral vascular involvement
          • Sickle-cell beta plus with cerebral vascular involvement
        • D57.458 Sickle-cell thalassemia beta plus with crisis with other specified complication

          Inclusion term(s):

          • HbS-beta plus with crisis with other specified complication
          • Sickle-cell beta plus with crisis with other specified complication
        • D57.459 Sickle-cell thalassemia beta plus with crisis, unspecified

          Inclusion term(s):

          • 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 vasoocclusive pain NOS
    6. D57.8 Other sickle-cell disorders

      Inclusion term(s):

      • Hb-SD disease
      • Hb-SE disease
      1. D57.80 Other sickle-cell disorders without crisis

      2. 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
        • D57.818 Other sickle-cell disorders with crisis with other specified complication
        • D57.819 Other sickle-cell disorders with crisis, unspecified

          Inclusion term(s):

          • Other sickle-cell disorders with crisis NOS
          • Other sickle-cell disorders with vasooclusive pain NOS
Abbreviations used here:

NEC Not elsewhere classifiable
This abbreviation in the Tabular List represents “other specified”. When a specific code is not available for a condition, the Tabular List includes an NEC entry under a code to identify the code as the “other specified” code.

NOS Not otherwise specified
This abbreviation is the equivalent of unspecified.