Oncology & Hematology Coding Alert

Hematology:

Are You Aware of All These Hemophilia Types? Find Out

Here’s what von Willebrand’s factor means.

In last month’s Oncology Coding Alert, you learned about “How to Collect the Clotting Factor You Deserve.” If you’re still uncertain about the types of hemophilia, you’re not alone. We have broken them down for you.

Hemophilia is a bleeding disorder characterized by deficiency of clotting factor in the blood. According to the Hemophilia Federation of America (URL: https://www.hemophiliafed.org/understanding-bleeding-disorders/what-is-hemophilia/), due to inheritance patterns, hemophilia mainly affects males. “Hemophilia affects 1 in 5,000 male births in the U.S. and approximately 400 babies are born with hemophilia each year,” according to the Hemophilia Federation of America. “400,000 people worldwide are living with hemophilia and about 20,000 are living with it in the United States alone. All races and economic groups are affected equally.”

Break Congenital Hemophilia into 2 Types

The most common types of congenital hemophilia are hemophilia A and B:

  • Hemophilia A is also called classic hemophilia. The cause of bleeding in this type of hemophilia is deficiency of factor VIII.
  • The next most common type of hemophilia is hemophilia B, also called Christmas disease (named for Stephen Christmas, the first patient described with hemophilia B). This type of hemophilia develops when factor IX is deficient. Congenital hemophilia is different from acquired hemophilia.

Acquired Hemophilia Develops in Adults

Acquired hemophilia, usually seen in adults, can develop consequent to illness, medications, or pregnancy. In this type of hemophilia, antibodies develop against clotting factors and these antibodies inactivate the clotting factors leading to bleeding. This type of hemophilia usually resolves when the antibody levels are controlled.

“Acquired hemophilia is not hereditary, this is an autoimmune disorder,” says Leah Fuller, CPC, COC, Consultant, Pinnacle Enterprise Risk Consulting Services, LLC, CO. “This means the body mistakenly attacks healthy tissue and cells, specifically clotting factors, which in turn leads to excessive bleeding. In some cases of acquired hemophilia, the patient has an underlying issue, in others, the cause is unknown. Congenital hemophilia is hereditary and is distinguished by a deficiency of certain clotting factors which occurs at birth. The provider should document in the medical record the type of hemophilia the patient has.”

Von Willebrand Factor has an important role to play to maintain the Factor VIII levels in the blood.

What is von Willebrand Factor? Von Willebrand Factor is a large protein which binds to clotting factor VIII to protect it and help platelets bind to injured blood vessels. It is used to help the body form blood clots.

“Von Willebrand Disease is the most common type of bleeding disorder, affecting an estimated 1% of the world’s population,” according to the Hemophilia Federation of America.