2021 ICD-10-CM Diagnosis Code D57

Sickle-cell disorders

    2016 2017 2018 2019 2020 2021 Non-Billable/Non-Specific Code
  • D57 should not be used for reimbursement purposes as there are multiple codes below it that contain a greater level of detail.
  • The 2021 edition of ICD-10-CM D57 became effective on October 1, 2020.
  • This is the American ICD-10-CM version of D57 - other international versions of ICD-10 D57 may differ.
Use Additional
Use Additional Help
Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology. For such conditions the ICD-10-CM has a coding convention that requires the underlying condition be sequenced first followed by the manifestation. Wherever such a combination exists there is a "use additional code" note at the etiology code, and a "code first" note at the manifestation code. These instructional notes indicate the proper sequencing order of the codes, etiology followed by manifestation. In most cases the manifestation codes will have in the code title, "in diseases classified elsewhere." Codes with this title are a component of the etiology/manifestation convention. The code title indicates that it is a manifestation code. "In diseases classified elsewhere" codes are never permitted to be used as first listed or principle diagnosis codes. They must be used in conjunction with an underlying condition code and they must be listed following the underlying condition.
  • code for any associated fever (
    ICD-10-CM Diagnosis Code R50.81

    Fever presenting with conditions classified elsewhere

      2016 2017 2018 2019 2020 2021 Billable/Specific Code Manifestation Code
    Code First
    • underlying condition when associated fever is present, such as with:
    • leukemia (C91-C95)
    • neutropenia (D70.-)
    • sickle-cell disease (D57.-)
    R50.81
    )
Type 1 Excludes
Type 1 Excludes Help
A type 1 excludes note is a pure excludes. It means "not coded here". A type 1 excludes note indicates that the code excluded should never be used at the same time as D57. A type 1 excludes note is for used for when two conditions cannot occur together, such as a congenital form versus an acquired form of the same condition.
  • other hemoglobinopathies (
    ICD-10-CM Diagnosis Code D58
    • D58 Other hereditary hemolytic anemias
      • D58.0 Hereditary spherocytosis
      • D58.1 Hereditary elliptocytosis
      • D58.2 Other hemoglobinopathies
      • D58.8 Other specified hereditary hemolytic anemias
      • D58.9 Hereditary hemolytic anemia, unspecified
    D58.-
    )
The following code(s) above D57 contain annotation back-references
Annotation Back-References
In this context, annotation back-references refer to codes that contain:
  • Applicable To annotations, or
  • Code Also annotations, or
  • Code First annotations, or
  • Excludes1 annotations, or
  • Excludes2 annotations, or
  • Includes annotations, or
  • Note annotations, or
  • Use Additional annotations
that may be applicable to D57:
  • D50-D89
    2021 ICD-10-CM Range D50-D89

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

    Type 2 Excludes
    • autoimmune disease (systemic) NOS (M35.9)
    • certain conditions originating in the perinatal period (P00-P96)
    • complications of pregnancy, childbirth and the puerperium (O00-O9A)
    • congenital malformations, deformations and chromosomal abnormalities (Q00-Q99)
    • endocrine, nutritional and metabolic diseases (E00-E88)
    • human immunodeficiency virus [HIV] disease (B20)
    • injury, poisoning and certain other consequences of external causes (S00-T88)
    • neoplasms (C00-D49)
    • symptoms, signs and abnormal clinical and laboratory findings, not elsewhere classified (R00-R94)
    Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism
Approximate Synonyms
  • Sickle cell nephropathy
Clinical Information
  • A blood disorder characterized by the appearance of sickle-shaped red blood cells and anemia.
  • A disease characterized by chronic hemolytic anemia, episodic painful crises, and pathologic involvement of many organs. It is the clinical expression of homozygosity for hemoglobin s.
  • An inherited disease in which the red blood cells have an abnormal crescent shape, block small blood vessels, and do not last as long as normal red blood cells. Sickle cell anemia is caused by a mutation (change) in one of the genes for hemoglobin (the substance inside red blood cells that binds to oxygen and carries it from the lungs to the tissues). It is most common in people of west and central african descent.
  • Disease characterized by chronic hemolytic anemia, episodic painful crises, and pathologic involvement of many organs; the clinical expression of homozygosity for hemoglobin s.
  • Sickle cell anemia is a disease in which your body produces abnormally shaped red blood cells. The cells are shaped like a crescent or sickle. They don't last as long as normal, round red blood cells, which leads to anemia. The sickle cells also get stuck in blood vessels, blocking blood flow. This can cause pain and organ damage. A genetic problem causes sickle cell anemia. People with the disease are born with two sickle cell genes, one from each parent. If you only have one sickle cell gene, it's called sickle cell trait. About 1 in 12 african americans has sickle cell trait. A blood test can show if you have the trait or anemia. Most states test newborn babies as part of their newborn screening programs.
Code History
  • 2016 (effective 10/1/2015): New code (first year of non-draft ICD-10-CM)
  • 2017 (effective 10/1/2016): No change
  • 2018 (effective 10/1/2017): No change
  • 2019 (effective 10/1/2018): No change
  • 2020 (effective 10/1/2019): No change
  • 2021 (effective 10/1/2020): No change
Code annotations containing back-references to D57:
  • Code First: R50.81
    , M14.8
    , H36
    ICD-10-CM Diagnosis Code R50.81

    Fever presenting with conditions classified elsewhere

      2016 2017 2018 2019 2020 2021 Billable/Specific Code Manifestation Code
    Code First
    • underlying condition when associated fever is present, such as with:
    • leukemia (C91-C95)
    • neutropenia (D70.-)
    • sickle-cell disease (D57.-)
    ICD-10-CM Diagnosis Code M14.8

    Arthropathies in other specified diseases classified elsewhere

      2016 2017 2018 2019 2020 2021 Non-Billable/Non-Specific Code
    Code First
    • underlying disease, such as:
    • amyloidosis (E85.-)
    • erythema multiforme (L51.-)
    • erythema nodosum (L52)
    • hemochromatosis (E83.11-)
    • hyperparathyroidism (E21.-)
    • hypothyroidism (E00-E03)
    • sickle-cell disorders (D57.-)
    • thyrotoxicosis [hyperthyroidism] (E05.-)
    • Whipple's disease (K90.81)
    ICD-10-CM Diagnosis Code H36

    Retinal disorders in diseases classified elsewhere

      2016 2017 2018 2019 2020 2021 Billable/Specific Code Manifestation Code
    Code First
    • underlying disease, such as:
    • lipid storage disorders (E75.-)
    • sickle-cell disorders (D57.-)
    Type 1 Excludes
  • Type 1 Excludes: D56.8
    ICD-10-CM Diagnosis Code D56.8

    Other thalassemias

      2016 2017 2018 2019 2020 2021 Billable/Specific Code
    Applicable To
    • Dominant thalassemia
    • Hemoglobin C thalassemia
    • Mixed thalassemia
    • Thalassemia with other hemoglobinopathy
    Type 1 Excludes
    • hemoglobin C disease (D58.2)
    • hemoglobin E disease (D58.2)
    • other hemoglobinopathies (D58.2)
    • sickle-cell anemia (D57.-)
    • sickle-cell thalassemia (D57.4)

Diagnosis Index entries containing back-references to D57:
  • Arthropathy M12.9
    - see also Arthritis
    ICD-10-CM Diagnosis Code M12.9

    Arthropathy, unspecified

      2016 2017 2018 2019 2020 2021 Billable/Specific Code
    • gouty - see also Gout
      • in (due to)
        • sickle-cell disorders D57-

ICD-10-CM Codes Adjacent To D57
D55.9 Anemia due to enzyme disorder, unspecified
D56 Thalassemia
D56.0 Alpha thalassemia
D56.1 Beta thalassemia
D56.2 Delta-beta thalassemia
D56.3 Thalassemia minor
D56.4 Hereditary persistence of fetal hemoglobin [HPFH]
D56.5 Hemoglobin E-beta thalassemia
D56.8 Other thalassemias
D56.9 Thalassemia, unspecified
D57 Sickle-cell disorders
D57.0 Hb-SS disease with crisis
D57.00 …… 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
D57.09 …… with other specified complication
D57.1 Sickle-cell disease without crisis
D57.2 Sickle-cell/Hb-C disease
D57.20 …… without crisis
D57.21 Sickle-cell/Hb-C disease with crisis

Reimbursement claims with a date of service on or after October 1, 2015 require the use of ICD-10-CM codes.