D66 - Hereditary factor VIII deficiency

Version 2023
Short Description:Hereditary factor VIII deficiency
Long Description:Hereditary factor VIII deficiency
Status: Valid for Submission
Version:ICD-10-CM 2023
Code Classification:
  • Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism (D50–D89)
    • Coagulation defects, purpura and other hemorrhagic conditions (D65-D69)
      • Hereditary factor VIII deficiency (D66)

D66 is a billable ICD-10 code used to specify a medical diagnosis of hereditary factor viii deficiency. The code is valid during the fiscal year 2023 from October 01, 2022 through September 30, 2023 for the submission of HIPAA-covered transactions.

Approximate Synonyms

The following clinical terms are approximate synonyms or lay terms that might be used to identify the correct diagnosis code:

Clinical Information

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 coding notes and guidance for inclusions, exclusions, descriptions and more. The following references are applicable to this diagnosis code:

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.

Type 1 Excludes

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.

Index to Diseases and Injuries References

The Index to Diseases and Injuries is an alphabetical listing of medical terms, with each term mapped to one or more ICD-10 code(s). The following references for this diagnosis code are found in the injuries and diseases index:

Convert to ICD-9 Code

Source ICD-10 CodeTarget ICD-9 Code
D66286.0 - Cong factor viii diord

Patient Education


What is hemophilia?

Hemophilia is a rare bleeding disorder in which the blood does not clot properly. This can lead to problems with bleeding too much after an injury or surgery. You can also have sudden bleeding inside your body, such as in your joints, muscles, and organs.

Your blood contains many proteins called clotting factors that can help form clots to stop bleeding. People with hemophilia have low levels of one of these factors, usually either factor VIII (8) or factor IX (9). How severe the hemophilia is depends on the amount of factor in the blood. The lower the amount of the factor, the more likely it is that bleeding could happen and might lead to serious health problems.

What are the types of hemophilia?

There are several different types of hemophilia. The most common are:

What causes hemophilia?

Most types of hemophilia are inherited. They are caused by change in one of the genes (also called a mutation) that provides instructions for making the clotting factor proteins. The change may mean that the clotting proteins don't work properly or that they are missing altogether.

These genes are on the X chromosome. You may have one or two X chromosomes:

Hemophilia that is not inherited is called acquired hemophilia. It is rare. It happens when your body makes specialized proteins called autoantibodies that attack and disable a clotting factor. This can happen because of pregnancy, immune system disorders, cancer, or allergic reactions to certain medicines. Sometimes the cause is unknown.

Who is at risk for hemophilia?

Hemophilia is much more common in people who were born male since they can get it with a change to the gene on one X chromosome. People who have a family history of hemophilia are also at higher risk.

What are the symptoms of hemophilia?

The signs and symptoms of hemophilia are:

In some cases, severe hemophilia may cause bleeding in the brain. This may cause brain damage and can be life-threatening.

How is hemophilia diagnosed?

To find out if you have hemophilia, your health care provider will:

There is genetic testing for the factor VIII (8) and factor IX (9) genes. This testing may be used in people who have a family history of hemophilia to:

What are the treatments for hemophilia?

The best way to treat hemophilia is to replace the missing clotting factor so that your blood can clot properly. This is usually done by injecting replacement clotting factor into a vein. The replacement clotting factor may be made from donated human blood. Or it may be made in a lab; this kind is called a recombinant clotting factor.

Replacement clotting factor can help treat a bleeding episode. In more severe cases of hemophilia, you might get the factor on a regular basis to prevent bleeding. You can learn how to inject the factor so that you can do it yourself at home.

There are other medicines to treat hemophilia. They may work by releasing factor VIII (8) from where it is stored in the body tissues, replacing the function of factor VIII (8), or preventing clots from breaking down.

If bleeding has damaged your joints, physical therapy may help them function better.

Good quality medical care from healthcare professionals who know a lot about the disorder can help prevent some serious problems. Often the best choice for care is to visit a hemophilia treatment center (HTC).

Centers for Disease Control and Prevention

[Learn More in MedlinePlus]


Hemophilia is a bleeding disorder that slows the blood clotting process. People with this condition experience prolonged bleeding or oozing following an injury, surgery, or having a tooth pulled. In severe cases of hemophilia, continuous bleeding occurs after minor trauma or even when there is no obvious injury (sometimes called spontaneous bleeding). Serious complications can result from bleeding into the joints, muscles, brain, or other internal organs. Milder forms of hemophilia do not necessarily involve spontaneous bleeding, and the condition may not become apparent until abnormal bleeding occurs following surgery or a serious injury.

The major types of this condition are hemophilia A (also known as classic hemophilia or factor VIII deficiency) and hemophilia B (also known as Christmas disease or factor IX deficiency). Although the two types have very similar signs and symptoms, they are caused by variants (also known as mutations) in different genes. People with an unusual form of hemophilia B, known as hemophilia B Leyden, experience episodes of excessive bleeding in childhood but have few bleeding problems after puberty.

[Learn More in MedlinePlus]

Code History