Condition
Comments
Surgical bleeding
Bleeding from a major artery or vein that was missed during surgery must be ruled out first, especially in the immediate postoperative period
Medications
Inquire about aspirin, clopidogrel, heparin, warfarin, or any other antiplatelet or anticoagulant medication
Inherited coagulation disorders
Patients with von Willebrand disease may have a history of excessive bleeding after minor procedures or very heavy menses; hemophilia A and B usually present in childhood with spontaneous hemorrhage into joints (hemarthrosis)
Liver disease
Reduced production of clotting factors
Renal failure
Uremia impairs platelet function
Disseminated intravascular coagulation (DIC)
Seen with severe sepsis, malignancy, and childbirth complications; leads to bleeding and microthrombi; manifests with diffuse bleeding from wounds and surgical sites, hematemesis, digital cyanosis, renal insufficiency, and stroke
The bloody vicious cycle (“lethal triad of death”)
Refers to three factors that work in concert: more common after long operations, trauma, large volume of room temperature IV fluids, and in those who have suffered significant bleeding
What Is the Most Likely Cause of the Bleeding in the Patient Described Above?
The bleeding is likely a medical bleed and not a surgical one. The finding of diffuse oozing at the time of operation together with a prolonged PTT would suggest an underlying bleeding diathesis.
What is the Differential Diagnosis for Prolonged PTT and the Common Features?
Condition | PT | PTT | BT | Acquired/congenital | History and physical |
|---|---|---|---|---|---|
Acquired FVIIII Inhibitors | − | ↑ | − | Acquired | Occurs in postpartum, rheumatic disease, and malignancy; presents with purpura and soft tissue bleeding |
Antiphospholipid syndrome (SLE)* | − | ↑ | − | Acquired | Young woman with malar rash, arthritis, photosensitivity, renal/cardiac symptoms, fevers, malaise, and recurrent pregnancy loss |
Hemophilia A | − | ↑ | − | Congenital | Presents early in childhood with spontaneous bleeding in joints (hemarthroses) or life-threatening hemorrhage following minor trauma |
Hemophilia B | − | ↑ | − | Congenital | Same as hemophilia A |
Heparin | − | ↑ | − | Acquired | Postoperative prophylaxis for DVT and PE, decreases post-MI thrombus risk |
Von Willebrand disease | − | −/↑ | ↑ | Both | Young woman with bleeding after minor surgical procedure or history of excessive menses |
What Is the Most Likely Diagnosis?
The above patient has an isolated prolonged PTT. Antiphospholipid syndrome associated with SLE is paradoxically a hypercoagulable state (see below) as opposed to causing bleeding. An acquired antibody to factor VIII (acquired hemophilia) is rare and is most commonly associated with postpartum patients, rheumatic disease, and cancer. Hemophilia A and B are clinically indistinguishable. With severe factor deficiencies, they present early in childhood with spontaneous bleeding in the joints (hemarthrosis) or life-threatening hemorrhage following trauma. Von Willebrand disease is not associated with a history of severe bleeding but rather with bleeding after minor surgical procedures or a history of excessive menses. Thus the most likely diagnosis is von Willebrand disease.
History and Physical Examination
Why Is It Important to Ask About a History of Bleeding After Minor Trauma/Procedures?
A history of bleeding suggests a predisposition to bleeding risk. Important questions to ask include a history of excessive bleeding in the mouth, epistaxis, bleeding into the muscle and joints, excessive menstrual bleeding, and excessive bleeding after minor procedures (dental extraction, skin biopsy).
Why Is It Important to Ask About a Family History of Bleeding?
A family history of bleeding suggests there may be an inherited bleeding disorder.
What Medical Conditions Are Risk Factors for Bleeding?
Liver and renal diseases, as well as nutritional deficiency, increase the risk of bleeding, the latter due to vitamin K deficiency. Malabsorption syndromes including short bowel syndrome and cystic fibrosis in particular lead to vitamin K deficiency. Cardiac disease, by virtue of the various antiplatelet agents often prescribed (aspirin, clopidogrel, warfarin), increases the risk of bleeding.
Physiology/Pathophysiology
What Is the Difference Between Primary and Secondary Hemostasis Disorders?
Disorders of primary hemostasis are usually due to abnormalities in platelets, whereas disorders of secondary hemostasis are usually due to factor abnormalities. Platelet abnormalities can be divided into quantitative or qualitative disorders. After primary hemostasis, the coagulation cascade (Fig. 38.1) generates thrombin, which converts fibrinogen in the platelet plug to fibrin. The fibrin is then cross-linked by factor VIII to form a stable platelet-fibrin thrombus. Impairment in this cascade can lead to disorders of secondary hemostasis and is most often due to factor abnormalities.
Fig. 38.1
Coagulation cascade. HMWK high molecular weight kininogen, PK prekallikrein, TF tissue factor (with kind permission from Springer Science + Business Media: Contemporary Cardiology: Antithrombotic Drug Therapy in Cardiovascular Disease, The Role of Coagulation in Arterial and Venous Thrombosis, 2010, pg 22, Kottke-Marchant K., Fig. 2)
What Is Coagulopathy?
This term is reserved for conditions that lead to an impairment of the body’s ability to clot blood. Normal blood clotting involves as many as 20 different plasma proteins. When these proteins are missing or deficient, patients can present with bleeding symptoms that can range from mild to severe. This can occur spontaneously or following minor trauma. Metabolic acidosis and hypothermia exacerbate coagulopathy.
What Is Meant by a Medical Versus a Surgical Postoperative Bleed?
A surgical bleed refers to bleeding that can be corrected with surgery, for example, bleeding from a focal area (an artery or vein) that was inadequately ligated or sutured during the initial surgery. A medical bleed refers to diffuse bleeding caused by underlying coagulopathy. Since medical bleeding is diffuse and caused by a bleeding disorder, reoperation is not beneficial.
What Is the Pathophysiology of Von Willebrand Disease (VWD)? What Are the Subtypes?
Von Willebrand factor is a protein needed to form a platelet plug. When vascular tissue is damaged, the exposed subendothelial collagen is able to bind to von Willebrand factor (VWF). Platelets can then bind to VWF using the GPIb receptor to ultimately form the platelet plug and thus complete primary hemostasis. VWD is most commonly congenital but can be acquired. The congenital form has 3 subtypes (Table 38.1) causing both qualitative and quantitative defects. Since VWF is also a cofactor for factor VIII, severely decreased levels of VWF can lead to abnormally prolonged PTT, depending on the degree of activity reduction of factor VIII.
Table 38.1
Subtypes of VWD
Type | Inheritance | Quantitative or qualitative | Features |
|---|---|---|---|
1 | AD | Quantitative | The most common overall, often has mild symptoms |
2 | AD | Both | Contains 4 subtypes of which type 2A is most common, often has moderate symptoms |
3 | AR | Quantitative
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