ConditionBlood & immune system
Bleeding Disorders: Types, Causes and Treatment
Bleeding disorders are a group of conditions in which the blood does not clot properly. They fall into three main groups: problems with clotting factors (such as hemophilia A and B), problems with von Willebrand factor (von Willebrand disease, the most common), and problems with platelets (too few or not working properly). Each can be inherited or acquired. Modern treatments, including factor replacement, desmopressin, tranexamic acid and newer non-factor therapies, allow most people to live full lives.
Also called: Coagulation disorders, clotting disorders, hemorrhagic disorders, inherited bleeding disorders
At a glance
- Main groups
- Clotting factor deficiencies, von Willebrand disease, platelet disorders, and blood vessel disorders.
- Most common inherited
- Von Willebrand disease (about 1 in 100), then hemophilia A.
- Most common acquired
- Blood thinners, liver disease, low platelets and vitamin K deficiency.
- Diagnosis
- Blood count, PT/INR, aPTT, then factor, VWF and platelet function tests.
- Treatments
- Desmopressin, tranexamic acid, factor concentrates, emicizumab, gene therapy for hemophilia, and platelet transfusion.
When to get help
Call 911 or go to the ER
- Any head injury, severe headache or vomiting in someone with a bleeding disorder
- Bleeding into the neck, throat or tongue, or difficulty breathing or swallowing
- Uncontrolled bleeding, vomiting blood or black stools
- A painful, swollen, hot joint or muscle in someone with hemophilia (treat with factor as instructed and seek care)
See a doctor within a day or two
- Signs of a new joint or muscle bleed: tingling, stiffness, warmth, reduced movement
- Many new bruises or a pinpoint purple rash
Book a routine appointment
- Planning surgery, dental work or pregnancy with a known bleeding disorder
- Relatives of someone with an inherited bleeding disorder, for testing or carrier screening
Main types of bleeding disorders
| Disorder | Problem | Inheritance | Typical bleeding | Main treatments |
|---|---|---|---|---|
| Von Willebrand disease | Low or faulty von Willebrand factor | Usually autosomal dominant; all sexes | Nosebleeds, heavy periods, bruising, dental bleeding | Desmopressin, tranexamic acid, VWF concentrates |
| Hemophilia A | Low factor VIII | X-linked; mainly males | Joint and muscle bleeds, prolonged bleeding after injury | Factor VIII, emicizumab, gene therapy |
| Hemophilia B | Low factor IX | X-linked; mainly males | As hemophilia A | Factor IX, gene therapy |
| Factor XI deficiency | Low factor XI | Autosomal; more common in Ashkenazi Jewish people | Bleeding after surgery or dental work | Tranexamic acid, plasma or factor XI |
| Rare factor deficiencies | Factors I, II, V, VII, X, XIII | Autosomal recessive | Varies; can be severe | Specific concentrates or plasma |
| Platelet function disorders | Platelets do not stick or release properly | Varies | Skin and mucous membrane bleeding | Desmopressin, tranexamic acid, platelet transfusion |
| Thrombocytopenia | Too few platelets | Mostly acquired | Petechiae, bruising, gum and nose bleeding | Treat cause; steroids, immunoglobulin for ITP |
Von Willebrand disease
Von Willebrand disease (VWD) is the most common inherited bleeding disorder. VWF helps platelets stick to injured vessels and protects factor VIII.
- Type 1: partial deficiency; about 3 in 4 cases; usually mild.
- Type 2: VWF does not work properly; several subtypes.
- Type 3: almost no VWF; rare and severe, with joint bleeding similar to hemophilia.
Many people, especially women with heavy periods, are undiagnosed.
Hemophilia
Hemophilia A (factor VIII) and B (factor IX) mainly affect males because the genes are on the X chromosome. Female carriers may have low factor levels and bleed more than usual.
Severity is classified by factor level:
- Severe: under 1% of normal; spontaneous bleeding into joints and muscles
- Moderate: 1% to 5%; bleeding after minor injuries
- Mild: 5% to 40%; bleeding mainly after surgery or major injury
Repeated joint bleeds cause hemophilic arthropathy (joint damage), which is why preventive treatment (prophylaxis) is standard in severe hemophilia.
Platelet disorders
Too few platelets
Immune thrombocytopenia (ITP) is an autoimmune condition destroying platelets. Other causes include leukemia, chemotherapy, liver disease with an enlarged spleen, viral infections and some medicines. See thrombocytopenia.
Platelets that do not work properly
Inherited platelet function disorders include Glanzmann thrombasthenia and Bernard-Soulier syndrome (rare, more severe) and milder storage pool and release disorders. Acquired platelet dysfunction is common with aspirin, clopidogrel, NSAIDs and kidney failure.
Acquired bleeding disorders
- anticoagulant and antiplatelet medicines
- liver disease
- vitamin K deficiency
- disseminated intravascular coagulation (DIC) in sepsis, trauma or pregnancy complications
- massive blood loss and transfusion
- acquired hemophilia (antibodies against factor VIII), often in older adults or after childbirth
See coagulopathy for how doctors assess these.
Vascular bleeding disorders
Some conditions cause bleeding because blood vessels are fragile rather than because of clotting problems: hereditary hemorrhagic telangiectasia, Ehlers-Danlos syndromes, scurvy and long-term steroid use.
Diagnosis
Signs that should prompt testing are described on our bleeding disorder symptoms page. Testing begins with a complete blood count, PT/INR, aPTT and fibrinogen, followed by specialist tests guided by the results:
| Result pattern | Possible causes |
|---|---|
| Low platelets | ITP, leukemia, liver disease, medicines |
| Prolonged aPTT, normal PT | Hemophilia A or B, factor XI deficiency, VWD (sometimes), heparin |
| Prolonged PT, normal aPTT | Factor VII deficiency, early liver disease, early vitamin K deficiency, warfarin |
| Both prolonged | Liver disease, DIC, vitamin K deficiency, factor II, V or X deficiency |
| All normal | VWD, platelet function disorders, factor XIII deficiency, vascular disorders |
Treatment
- Desmopressin: a nasal spray or injection that raises VWF and factor VIII in responsive people.
- Antifibrinolytics: tranexamic acid or aminocaproic acid stabilize clots; very useful for mouth, nose and menstrual bleeding.
- Factor concentrates: plasma-derived or recombinant; standard and extended half-life versions.
- Non-factor therapies for hemophilia: emicizumab (a weekly or less frequent injection that mimics factor VIII) and newer rebalancing agents.
- Gene therapy: one-time treatments approved for some adults with severe hemophilia A or B.
- Platelet transfusions and, for ITP, steroids, immunoglobulin and thrombopoietin receptor agonists.
- Supportive care: iron for anemia, hormonal treatment for heavy periods, physiotherapy for joint health.
Care at a specialist hemophilia treatment center improves outcomes for moderate and severe disorders.
Frequently asked questions
What are the main types of bleeding disorders?
The main types are von Willebrand disease, hemophilia A and B, other clotting factor deficiencies, platelet disorders (too few or poorly working platelets) and acquired disorders caused by medicines, liver disease, vitamin K deficiency or DIC.
What is the difference between hemophilia and von Willebrand disease?
Hemophilia is a lack of factor VIII or IX, mostly affecting males and causing joint and muscle bleeds. Von Willebrand disease is a lack or malfunction of von Willebrand factor, affects all sexes, is more common, and usually causes nosebleeds, bruising and heavy periods.
Are bleeding disorders inherited?
Many are. Hemophilia is X-linked, von Willebrand disease is usually autosomal dominant, and rarer factor deficiencies are usually autosomal recessive. Bleeding disorders can also be acquired through medicines, liver disease, vitamin K deficiency and autoimmune conditions.
Can bleeding disorders be cured?
Most inherited bleeding disorders are lifelong, but treatment controls bleeding very effectively. Gene therapy now offers long-lasting factor production for some adults with hemophilia. Acquired disorders can often be corrected by treating the cause or stopping a medicine.
How are bleeding disorders treated during surgery?
A hematologist plans treatment in advance, which may include desmopressin, factor concentrates, tranexamic acid or platelet transfusions before and after the procedure. Always tell your surgeon and dentist about your bleeding disorder well ahead of any procedure.
Sources
This guide was written from current patient and clinical guidance, including:
- National Heart, Lung, and Blood Institute: Bleeding disorders
- World Federation of Hemophilia: Guidelines for the management of hemophilia (3rd edition)
- CDC: Hemophilia; Von Willebrand disease
- National Bleeding Disorders Foundation: Types of bleeding disorders
- ASH ISTH NHF WFH 2021 guidelines on the management of von Willebrand disease