Hemophilia A: Understanding The ICD-10 Code And Implications For Diagnosis

What is Hemophilia A?

Hemophilia A is a genetic disorder that impairs the body’s ability to control blood clotting. It is caused by a deficiency or dysfunction of factor VIII, a protein necessary for blood clotting. This condition results in prolonged bleeding and easy bruising, as blood does not clot properly.

Code Information

Coagulation Disorders: Hereditary and Hemorrhagic Disorders
Coagulation Disorders: Hereditary and Hemorrhagic Disorders

The ICD-10 code for Hemophilia A is D66. Hemophilia A is classified under the category of coagulation defects, purpura, and other hemorrhagic conditions in the ICD-10 coding system.

Diagnostic Related Groups (MS-DRG)

ICD--CM Diagnosis Code D
ICD–CM Diagnosis Code D

In the Medicare Severity-Diagnosis Related Group (MS-DRG) system, Hemophilia A falls under DRG 811 – Red Blood Cell Disorders with MCC (major complications or comorbidities) or DRG 812 – Red Blood Cell Disorders without MCC.

Convert to ICD-9 Code

For those still using the ICD-9 coding system, the equivalent code for Hemophilia A is 286.0.

Code History

The ICD-10 code for Hemophilia A, D66, was introduced in October 2015 as part of the tenth revision of the International Classification of Diseases.

Approximate Synonyms

Other names and terms used to refer to Hemophilia A include Hemophilic Christmas disease, Classic hemophilia, and Factor VIII deficiency.

Clinical Information

Hemophilia A is an X-linked recessive disorder, meaning it primarily affects males. Symptoms typically manifest in childhood and may include frequent nosebleeds, easy bruising, and prolonged bleeding from minor injuries or surgeries.


Hemophilia A is caused by a mutation in the F8 gene, which encodes factor VIII. This mutation results in reduced or dysfunctional factor VIII protein, leading to impaired blood clotting.


Common symptoms of Hemophilia A include excessive bleeding from cuts or injuries, easy bruising, joint pain and swelling, and prolonged bleeding after dental work or surgery.


Diagnosis of Hemophilia A is typically confirmed through blood tests that measure clotting factors, specifically factor VIII levels. Genetic testing may also be conducted to identify the specific mutation causing the disorder.


Management of Hemophilia A includes replacement therapy with recombinant factor VIII to restore blood clotting function. Other treatments may include desmopressin therapy, which can temporarily increase factor VIII levels, and prophylactic treatment to prevent bleeding episodes.


Overall, Hemophilia A is a complex genetic disorder that affects blood clotting and can lead to serious complications if not properly managed. Understanding the ICD-10 code and associated clinical information is essential for accurate diagnosis and treatment of this condition.


1. Is Hemophilia A a life-threatening condition?
2. Can females be carriers of the Hemophilia A gene?
3. What are the complications of Hemophilia A?
4. How is Hemophilia A inherited?
5. What advancements have been made in the treatment of Hemophilia A?

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