The bleeding history is where a bleeding disorder is first suspected or set aside. A detailed history matters because it gives clues about three things: how long the condition has lasted, whether it is inherited or acquired, and which part of hemostasis is at fault, judged from the site of bleeding — mucosa or joint on one side, an arterial or venous thrombosis on the other.
Past bleeding
A history of bleeding in the past, especially bleeding provoked by trauma or surgery, is one of the most useful questions about the condition. The bleeding symptoms that most often bring a patient to the clinic are prolonged bleeding after surgery, after a dental procedure, or after trauma, heavy menstrual bleeding, and heavy bleeding after childbirth.
New-onset bleeding without a previous history
Bleeding that appears for the first time in a patient with no earlier history points to an acquired disorder, such as immune thrombocytopenia, or ITP, acute leukemia, acquired hemophilia and disseminated intravascular coagulation, or DIC.
Family history
A lack of family history does not rule out the inherited bleeding disorders. About 30 to 40% of hemophilia A cases arise from a de novo mutation, and an autosomal recessive condition such as Glanzmann thrombasthenia may have no affected relatives at all. Inherited bleeding disorders, if they are very severe, show themselves in childhood; others, such as von Willebrand disease (VWD), mostly present in adulthood.
Easy bruising
Large and easy bruising is a common complaint that often has no pathological basis. When it does have one, the causes include bleeding disorders; disorders of the blood vessels and their supporting connective tissue, such as Ehlers-Danlos syndrome; Cushing’s syndrome; and prolonged use of corticosteroids. Senile purpura — easy bruising of the skin after minor trauma — is a separate age-related pattern.
A bruise can also come from a skin disorder rather than from a bleeding disorder. The distribution and pattern of a bruise carries information: bruises limited to the hand and forearm suggest senile purpura, while bruises in other distributions raise concern about elder abuse or intimate partner violence. Heavy menstrual bleeding and easy bruising are both common in patients with and without a bleeding disorder, so a positive complaint on its own proves little.
Spontaneous and physiological heavy bleeding
The site of spontaneous bleeding points to the part of hemostasis at fault, starting with the joints.
Spontaneous hemarthroses, or joint bleeding, are an important sign of moderate to severe factor VIII or factor IX deficiency. They occur very rarely except in von Willebrand disease, or VWD, when the related factor VIII level is less than 5% of its normal value. Hemarthroses and spontaneous muscle hematomas are characteristics of congenital moderate to severe deficiency of factor VIII and IX; they can also be seen in moderate to severe deficiency of fibrinogen, prothrombin, factor V, VII and X.
Spontaneous mucosal bleeding symptoms are an important sign of a platelet disorder or von Willebrand disease, or VWD — in other words, of a primary hemostasis disorder.
Epistaxis, or spontaneous bleeding of the nose, is particularly common in children and in dry climates. It may reflect no disorder at all; it may be the presenting symptom of VWD, especially in boys; and it is the most common symptom of hereditary hemorrhagic telangiectasia, or HHT.
Heavy menstrual bleeding is defined as bleeding more than 80 ml of blood in each cycle. In practice it is seen as the passage of clots more than 2.5 cm in diameter, the need to change a tampon more than hourly, or menses lasting more than 8 days, and it can result in iron-deficiency anemia. It is common in women with bleeding disorders such as VWD — where oral contraceptives or hormone replacement therapy increase the formation of VWF — factor XI deficiency, platelet function disorders and hemophilia.
Procedure-induced bleeding
The bleeding that follows a specific procedure is often the first clue, and the pattern points to particular defects.
- Bleeding with eruption of the primary teeth is seen in children with severe bleeding disorders, such as moderate to severe hemophilia; it is uncommon in children with mild bleeding disorders.
- Bleeding during a gum-involving procedure, such as cleaning the teeth, is more common in patients with a platelet adhesion disorder.
- Postpartum heavy bleeding: in women with type 1 VWD or hemophilia A, supplementing VWF and factor VIII during the pregnancy decreases the chance of bleeding during delivery. Bleeding is more common 24 to 48 hours after delivery, because in the second and third trimester the estrogen improves hemostasis by increasing the production of VWF.
- Tonsillectomy: in patients with bleeding disorders, it is possible to see heavy bleeding after the procedure, or a delayed bleeding about 7 days after it.
- Delayed post-operation bleeding can be a sign of factor XIII deficiency.
Drug- or supplement-related bleeding
Drugs that increase bleeding act at different points in hemostasis.
- NSAIDs inhibit the COX-1 enzyme, and so they exacerbate the bleeding and also unmask mild bleeding disorders such as VWD. The effect of aspirin on the platelets lasts for the life of the platelet, which in normal conditions is about 7 to 10 days.
- P2Y12 inhibitors inhibit platelet aggregation. They are clopidogrel and ticagrelor, and the risk of bleeding with these drugs is higher than with NSAIDs.
- Vitamin K inhibitors such as warfarin block the factors activated by vitamin K, which are factor II, factor VII, factor IX, factor X, protein C, protein S and protein Z.
- Oral contraceptives and hormone replacement therapy increase the formation of VWF and therefore improve hemostasis, which matters when a woman’s bleeding history is assessed.
Herbal supplements can also contribute. Fish oil, or concentrated omega-3 products, causes the production of more PGI3 than PGI2, or prostacyclin, which is a more potent platelet inhibitor, and the production of more TxA3 than TxA2, which is a less potent platelet activator. Vitamin E inhibits protein kinase C, platelet aggregation and nitric oxide production.
Bleeding secondary to a systemic disorder
Several systemic illnesses cause bleeding, and recognising them changes management. The main ones are:
- hepatic failure, which leads to combined coagulation factor deficiencies; the bleeding it produces is accompanied by thrombocytopenia and portal hypertension;
- renal failure;
- cancer;
- hypothyroidism;
- amyloidosis;
- connective tissue disorders such as lupus;
- bone marrow failure.
Prolonged tobacco smoking also increases the chance of a bleeding disorder, and the same applies to excessive alcohol use.
Life-threatening sites of bleeding
Three sites of bleeding demand immediate attention because they kill quickly:
- the oropharynx, because the blood will enter the airway and stop the patient from breathing
- the CNS, which is the major cause of death in patients with severe congenital factor deficiencies
- the retroperitoneum
Bleeding patterns by the part of hemostasis involved
The pattern of bleeding is a guide to which part of hemostasis is defective.
- Primary hemostasis-related bleeding disorders often present with mucocutaneous bleeding and petechiae.
- Secondary hemostasis-related bleeding disorders often present with hematoma, mainly in muscles, hemarthroses, and bleeding during circumcision, which is a sign of severe hemophilia A or B. Two factors are exceptions: factor XI deficiency causes mucosal bleeding, which otherwise belongs to the primary hemostasis pattern, and factor XIII deficiency causes delayed post-surgery bleeding and umbilical stump bleeding.
- Fibrinolysis-related disorders cause delayed bleeding after trauma or surgery.
- Platelet or vascular disorders often present with mucosal bleeding.