Hemophilia and Bleeding Disorders

This set of vocabulary flashcards covers the pathophysiology, types, genetics, symptoms, diagnosis, and management of Hemophilia as discussed in the medical-surgical series.

Hemophilia

An X-linked genetic disorder affecting the sex chromosome X, resulting in a deficiency of certain clotting factors and prolonged bleeding.

Platelets

Blood components responsible for the temporary stoppage of bleeding during clot formation.

Clotting Factors

Proteins responsible for creating the permanent seal over a clot to effectively stop bleeding.

Hemophilia A

A deficiency in clotting factor $8$, which is the most common type occurring in one in every $5000$ births.

Hemophilia B

A deficiency in clotting factor $9$, also known as Christmas disease.

Christmas Disease

Another name for Hemophilia B, named because clotting factor $9$ is known as the Christmas factor.

Hemophilia C

A deficiency in clotting factor $11$.

X-linked Disorder

A genetic condition where females typically act as carriers due to having two X chromosomes, while males manifest the condition because they have only one X chromosome.

Epistaxis

Medical term for a nosebleed, which occurs in hemophilia patients due to the rupture of fragile small blood vessels in the nasal passage.

Hemarthrosis

Recurrent bleeding into the joints, most commonly occurring in the knees, elbows, wrists, and shoulders.

Ankylosis

Joint stiffness and damage resulting from repeated inflammation and hemarthrosis over time.

Hematoma

Bleeding deep into the muscles that can cause pressure-related nerve damage and muscle atrophy.

Factor Concentrates

Specific clotting factor replacement therapy preferred over fresh frozen plasma to avoid fluid overload.

Plasmapheresis

A procedure where blood is cycled through a machine to remove antibodies formed against factor concentrates.

DDAVP (Desmopressin)

The drug of choice for Hemophilia A that induces the body to produce significant amounts of clotting factor $8$.

RICE

A protocol for managing acute injury consisting of Rest, Ice, Compress, and Elevate; ice is applied for the first $24$ hours to constrict blood vessels.

Bleeding Precautions

Safety measures for hemophilia patients including avoiding contact sports, using electric razors, and avoiding medications like aspirin or ibuprofen.