There are numerous types of bleeding disorders that make up the fabric of our community. Below we have summarized the different types of bleeding disorders, but if you would like more in depth information about any individual bleeding disorder, or the effects of inhibitors we encourage you to explore the resources availabe on the World Federation of Hemophilia's eLearning Centers website

Type of Bleeding Disorders

Brief Description

Common Signs and Symptoms

Known Treatments

Von Willebrand Disease

vWD is the most common inherited bleeding disorder.  As many as 1 in every 100 people has it and it effects men and women equally.  The von Willebrand factor is a protein in the blood and those with this disease have low levels of this factor or normal levels but their factor is defective.  This interferes with normal blood clotting in the patient.

 

-     Easy bruising

-     Bleeding from the nose, gums, or mouth

-     Upset stomach

-     Vomit that is black and syrupy or bright red

-     Red or black colored stool

-     Bleeding after surgery

-     In women, heavy menstrual bleeding

-    Desmopressin

-    Factor concentrates

-    Hormone treatment

 

Bleeding can sometimes be controlled by drugs such as:

Tranexamic acid, aminocaproic acid, or

fibrin glue

Factor I (fibrinogen) deficiencies

 

1.

Afibrinogenemia

 

 

 

 

 

2. Hypofibrinogenemia

 

 

 

 

 

3. Dysfibrinogenemia

This refers to a problem with a protein called fibrinogen which helps

produce another protein, fibrin, which is needed to form a stable clot.

Patients have no factor I in their blood and therefore cannot make fibrin and form a stable clot.  This is inherited and can occur in both men and women

-    Nosebleeds & mouth bleeds

-    Easy bruising

-    Heavy or prolonged menstrual bleeding

-    Muscle bleeds

-    Bleeding into joints

-    Abnormal bleeding during or after injury

-    Problems during pregnancy

 

-    Fibrinogen concentrate

-    Cryoprecipitate

-    Fresh frozen plasma

Patients have lower than normal levels of fibrinogen so they cannot make fibrin or form a stable clot.  This is inherited from both parents and occurs equally in men and women

-     In women, heavy menstrual bleeding

-     Muscle ache

-     Tight and shiny appearance of skin

-     Upset stomach

-     Vomit that is black and syrupy or bright red

-     Red or black colored stool

 

-    Fibrinogen concentrate

-    Cryoprecipitate

-    Fresh frozen plasma

These patients have fibrinogen but it does not work as it should.  It is usually inherited from one parent and can occur in men and women equally.  It can also develop as a person gets older

-     Delayed wound healing

-     Rupture of medical sutures

-     Dead or dying skin

-     In women, miscarriage, stillbirths, and excessive bleeding following childbirth

-     Blood clots

 

-    Fibrinogen concentrate

-    Cryoprecipitate

-    Fresh frozen plasma

Factor II deficiency

Patients do not have enough factor II, or prothrombin, needed to help change fibrinogen into fibrin and form a stable clot.  Inherited from both parents and can occur in men and women.  It is one of the rarest inherited bleeding disorders, occurring in 1 in every 2 million people.

-     Bleeding into joints

-     Easy bruising

-     Bleeding from the nose, gums, or uterus

-     Bleeding after surgery

-     Nosebleeds

-     Muscle bleeds

-     Heavy or prolonged menstrual bleeding

 

-    Prothrombin complex concentrates (PCCs)

-    Fresh frozen plasma (FFP)

Factor V deficiency

These patients have low levels of factor V which helps produce thrombin and without it the body cannot form a stable clot to stop the bleed.  It is inherited from both parents and can occur in men and women, or develop as a person gets older. 

 

-     Bruises that appear easily

-     Bleeding from the mouth or nose

-     In women, heavy menstrual bleeding

-    Fresh frozen plasma (FFP)

-    Usually only needed for severe bleeds or before surgery

Factor VII deficiency

Patients have low levels of factor VII which plays an important role in starting the blood-clotting process.  One of the most common bleeding disorders, occurring in 1 in every 300,000-500,000 people.  It is inherited from both parents and occurs in men and women equally. 

-     Nosebleeds

-     Easy bruising

-     Heavy or prolonged menstrual bleeding

-     Bleeding in the mouth, especially after dental surgery or tooth extraction

-     Bleeding in the head (newborns)

-     Heavy bleeding at circumcision

 

-    Recombinant VIIa concentrate (rFVIIa)

-    Factor VII concentrate

-    Prothrombin complex concentrate (PCC) containing factor VII

-    Fresh frozen plasma (FFP)

Factor VIII deficiency

Sometimes called hemophilia A, people have low levels of factor VIII and without it the body cannot form a stable clot.  Affects mostly males who inherit the gene from their mothers.  It occurs in 1 in 5000 male births.

-     Big bruises

-     Bleeding into muscles and joints

-     Spontaneous bleeding

-     Prolonged bleeding after getting a cut, removing a tooth, or having surgery

-     Bleeding for a long time after an accident

 

-    Factor VIII concentrate

-    Fresh frozen plasma (FFP)

-    Desmopressin

Factor IX deficiency

Also known a hemophilia B and those with it do not have enough factor IX in their blood and therefore cannot form a stable clot.  It is inherited and affects mostly males, occurring in 1 in every 30,000 births.

-     Big bruises

-     Bleeding into muscles and joints

-     Spontaneous bleeding

-     Prolonged bleeding after getting a cut, removing a tooth, or having surgery

-     Bleeding for a long time after an accident

-    Factor concentrates

-    Fresh frozen plasma (FFP)

Factor X deficiency

Factor X helps activate prothrombin in patients, which helps form blood clots, so a lack of it causes patients to have trouble forming a stable blood clot.  It is inherited from both parents or can develop as a person gets older.  It occurs in 1 in every 500,000 to 1 million people.

 

-     Nose and mouth bleeds

-     Easy bruising

-     Joint and muscle bleeds

-     GI bleeding

-     Umbilical cord bleeding

-     Bleeding from surgery

-    Prothrombin complex concentrate (PCC) containing factor X

-    Fresh frozen plasma (FFP)

Factor XI deficiency

This is also called hemophilia C, and factor XI is not as critical to clotting as the other factors, but those with a deficiency may still need treatment and attention.  If either parent has factor XI deficiency it will be passed on to their children. It occurs in 1 in every 1 million people.

 

-     Nosebleeds

-     Easy bruising

-     Heavy or prolonged menstrual bleeding

-     Abnormal bleeding during or after surgery, injury, or childbirth

-    Factor XI concentrate

-    Antifibrinolytic drugs

-    Fibrin glue

-    Fresh frozen plasma (FFP)

Factor XIII deficiency

Factor XIII helps end the blood-clotting process by helping fibrin link together, so without enough factor XIII a stable clot cannot be formed.  It is usually inherited from both parents but can develop as a person gets older.  It can occur in both men and women and it occurs in 1 in every 3-5 million people

-     Bleeding from the umbilical cord

-     Nosebleeds

-     Easy bruising

-     Bleeding into joints & soft tissue

-     Bleeding into the central nervous system

-     Bleeding in the mouth

-     Poor wound healing and abnormal scar formation

-     problems during pregnancy

 

-    factor XIII concentrate

-    Cryoprecipitate

-    Fresh frozen plasma (FFP)

Platelet Disorders              Platelet- tiny cells in the blood that stick to an injured blood vessel, and to each other, to form a plug

Glanzmann thrombasthenia

Patients have a defective glycoprotein or no glycoprotein, which is a protein that helps platelets work properly by acting as a bridge to form a plug and stop bleeding.  It is inherited from both parents and can occur in men and women

 

-     Excessive bleeding after surgery or injury

-     Easy bruising

-     Bleeding from the nose or gums

-     In women, heavy menstruation

-    Antifibrinolytic drugs

-    Recombinant factor VIIa

-    Fibrin sealants

-    Hormonal contraceptives

-    Iron replacement

-    Platelet transfusions

Bernard-Soulier syndrome

Patients have a defective glycoprotein causing platelets to either be abnormally large, not stick to each other, or they may not have enough to stop the bleeding.  This is inherited from both parents and can occur in both men and women

-     Easy bruising

-     Nose bleeds

-     Bleeding from gums

-     Heavy or prolonged menstrual bleeding or bleeding after child birth

-     Abnormal bleeding after surgery

 

-    Antifibrinolytic drugs

-    Recombinant factor VIIa

-    Desmopressin

-    Fibrin sealants

-    Hormonal contraceptives

-    Iron replacement

-    Platelet transfusions

Platelet storage pool deficiency

Platelets need the help of granules to carry signals from one end of a platelet to the other and when they don’t work properly the platelets cannot form a plug to stop bleeding.  It is inherited from one or both parents and can occur in men and women

 

-     Easy bruising

-     Nose bleeds

-     Bleeding from gums

-     Heavy or prolonged menstrual bleeding or bleeding after childbirth

-     Abnormal bleeding after surgery

-    Antifibrinolytic drugs

-    Desmopressin

-    Platelet transfusions

  


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