ICD-10-CM Code D57.4

Sickle-cell thalassemia

Version 2021 Non-Billable Code

Not Valid for Submission

D57.4 is a "header" nonspecific and non-billable code code, consider using a code with a higher level of specificity for a diagnosis of sickle-cell thalassemia. The code is NOT valid for the year 2021 for the submission of HIPAA-covered transactions.

ICD-10:D57.4
Short Description:Sickle-cell thalassemia
Long Description:Sickle-cell thalassemia

Consider the following ICD-10 codes with a higher level of specificity:

  • D57.40 - Sickle-cell thalassemia without crisis
  • D57.41 - ... unspecified, with crisis
  • D57.411 - ... unspecified, with acute chest syndrome
  • D57.412 - ... unspecified, with splenic sequestration
  • D57.413 - ... unspecified, with cerebral vascular involvement
  • D57.418 - ... unspecified, with crisis with other specified complication
  • D57.419 - ... 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
  • D57.438 - 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
  • D57.458 - Sickle-cell thalassemia beta plus with crisis with other specified complication
  • D57.459 - Sickle-cell thalassemia beta plus with crisis, unspecified

Tabular List of Diseases and Injuries

The Tabular List of Diseases and Injuries is a list of ICD-10 codes, organized "head to toe" into chapters and sections with guidance for inclusions, exclusions, descriptions and more. The following references are applicable to the code D57.4:

Inclusion Terms

Inclusion Terms
These terms are the conditions for which that code is to be used. The terms may be synonyms of the code title, or, in the case of "other specified" codes, the terms are a list of the various conditions assigned to that code. The inclusion terms are not necessarily exhaustive. Additional terms found only in the Alphabetic Index may also be assigned to a code.
  • Sickle-cell beta thalassemia
  • Thalassemia Hb-S disease

Code Classification

  • 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)

Code History

  • FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016
    (First year ICD-10-CM implemented into the HIPAA code set)
  • FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
  • FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
  • FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
  • FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020
  • FY 2021 - No Change, effective from 10/1/2020 through 9/30/2021

Information for Patients


Sickle Cell Disease

Also called: Hemoglobin SS disease, Sickle cell anemia

What is sickle cell disease?

Sickle cell disease (SCD) is a group of inherited red blood cell disorders. If you have SCD, there is a problem with your hemoglobin. Hemoglobin is a protein in red blood cells that carries oxygen throughout the body. With SCD, the hemoglobin forms into stiff rods within the red blood cells. This changes the shape of the red blood cells. The cells are supposed to be disc-shaped, but this changes them into a crescent, or sickle, shape.

The sickle-shaped cells are not flexible and cannot change shape easily. Many of them burst apart as they move through your blood vessels. The sickle cells usually only last 10 to 20 days, instead of the normal 90 to 120 days. Your body may have trouble making enough new cells to replace the ones that you lost. Because of this, you may not have enough red blood cells. This is a condition called anemia, and it can make you feel tired.

The sickle-shaped cells can also stick to vessel walls, causing a blockage that slows or stops the flow of blood. When this happens, oxygen can't reach nearby tissues. The lack of oxygen can cause attacks of sudden, severe pain, called pain crises. These attacks can occur without warning. If you get one, you might need to go to the hospital for treatment.

What causes sickle cell disease?

The cause of SCD is a defective gene, called a sickle cell gene. People with the disease are born with two sickle cell genes, one from each parent.

If you are born with one sickle cell gene, it's called sickle cell trait. People with sickle cell trait are generally healthy, but they can pass the defective gene on to their children.

Who is at risk for sickle cell disease?

In the United States, most of the people with SCD are African Americans:

  • About 1 in 13 African American babies is born with sickle cell trait
  • About 1 in every 365 black children is born with sickle cell disease

SCD also affects some people who come from Hispanic, southern European, Middle Eastern, or Asian Indian backgrounds.

What are the symptoms of sickle cell disease?

People with SCD start to have signs of the disease during the first year of life, usually around 5 months of age. Early symptoms of SCD may include

  • Painful swelling of the hands and feet
  • Fatigue or fussiness from anemia
  • A yellowish color of the skin (jaundice) or the whites of the eyes (icterus)

The effects of SCD vary from person to person and can change over time. Most of the signs and symptoms of SCD are related to complications of the disease. They may include severe pain, anemia, organ damage, and infections.

How is sickle cell disease diagnosed?

A blood test can show if you have SCD or sickle cell trait. All states now test newborns as part of their screening programs, so treatment can begin early.

People who are thinking about having children can have the test to find out how likely it is that their children will have SCD.

Doctors can also diagnose SCD before a baby is born. That test uses a sample of amniotic fluid (the liquid in the sac surrounding the baby) or tissue taken from the placenta (the organ that brings oxygen and nutrients to the baby).

What are the treatments for sickle cell disease?

The only cure for SCD is bone marrow or stem cell transplantation. Because these transplants are risky and can have serious side effects, they are usually only used in children with severe SCD. For the transplant to work, the bone marrow must be a close match. Usually, the best donor is a brother or sister.

There are treatments that can help relieve symptoms, lessen complications, and prolong life:

  • Antibiotics to try to prevent infections in younger children
  • Pain relievers for acute or chronic pain
  • Hydroxyurea, a medicine that has been shown to reduce or prevent several SCD complications. It increases the amount of fetal hemoglobin in the blood. This medicine is not right for everyone; talk to your health care provider about whether you should take it. This medicine is not safe during pregnancy.
  • Childhood immunizations to prevent infections
  • Blood transfusions for severe anemia. If you have had some serious complications, such as a stroke, you may have transfusions to prevent more complications.

There are other treatments for specific complications.

To stay as healthy as possible, make sure that you get regular medical care, live a healthy lifestyle, and avoid situations that may set off a pain crisis.

NIH: National Heart, Lung, and Blood Institute

  • Hemoglobin electrophoresis (Medical Encyclopedia)
  • Sickle cell anemia (Medical Encyclopedia)
  • Sickle cell test (Medical Encyclopedia)

[Learn More]

Thalassemia

Also called: Cooley's anemia, Mediterranean anemia

Thalassemias are inherited blood disorders. If you have one, your body makes fewer healthy red blood cells and less hemoglobin. Hemoglobin is a protein that carries oxygen to the body. That leads to anemia. Thalassemias occur most often among people of Italian, Greek, Middle Eastern, Southern Asian, and African descent.

Thalassemias can be mild or severe. Some people have no symptoms or mild anemia. The most common severe type in the United States is called Cooley's anemia. It usually appears during the first two years of life. People with it may have severe anemia, slowed growth and delayed puberty, and problems with the spleen, liver, heart, or bones.

Doctors diagnose thalassemias using blood tests. Treatments include blood transfusions and treatment to remove excess iron from the body. If you have mild symptoms or no symptoms, you may not need treatment. In some severe cases, you may need a bone marrow transplant.

NIH: National Heart, Lung, and Blood Institute

  • Thalassemia (Medical Encyclopedia)

[Learn More]

Sickle cell disease Sickle cell disease is a group of disorders that affects hemoglobin, the molecule in red blood cells that delivers oxygen to cells throughout the body. People with this disease have atypical hemoglobin molecules called hemoglobin S, which can distort red blood cells into a sickle, or crescent, shape.Signs and symptoms of sickle cell disease usually begin in early childhood. Characteristic features of this disorder include a low number of red blood cells (anemia), repeated infections, and periodic episodes of pain. The severity of symptoms varies from person to person. Some people have mild symptoms, while others are frequently hospitalized for more serious complications.The signs and symptoms of sickle cell disease are caused by the sickling of red blood cells. When red blood cells sickle, they break down prematurely, which can lead to anemia. Anemia can cause shortness of breath, fatigue, and delayed growth and development in children. The rapid breakdown of red blood cells may also cause yellowing of the eyes and skin, which are signs of jaundice. Painful episodes can occur when sickled red blood cells, which are stiff and inflexible, get stuck in small blood vessels. These episodes deprive tissues and organs, such as the lungs, kidneys, spleen, and brain, of oxygen-rich blood and can lead to organ damage. A particularly serious complication of sickle cell disease is high blood pressure in the blood vessels that supply the lungs (pulmonary hypertension), which can lead to heart failure. Pulmonary hypertension occurs in about 10 percent of adults with sickle cell disease.
[Learn More]