Sickle-cell disorders D57-

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 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.-)
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
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.
  • 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
    • D57.1 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
        • D57.211 Sickle-cell/Hb-C disease with acute chest syndrome
        • D57.212 Sickle-cell/Hb-C disease with splenic sequestration
        • D57.219 …… unspecified
    • D57.3 Sickle-cell trait
    • D57.4 Sickle-cell thalassemia
      • D57.40 …… without crisis
      • D57.41 Sickle-cell thalassemia with crisis
        • D57.411 Sickle-cell thalassemia with acute chest syndrome
        • D57.412 Sickle-cell thalassemia with splenic sequestration
        • D57.419 …… unspecified
    • D57.8 Other sickle-cell disorders
      • D57.80 …… 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
        • D57.819 …… unspecified