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Unexplained Bleeding in the Emergency Department

A patient presents in the emergency department (ED) with a nose bleed that won’t stop or a menstrual period lasting several weeks. Although bleeding disorders are rare, they often first present to the ED.

Bleeding disorders encompass many conditions that are a result of some type of blood clotting issue. There are 2 types of bleeding disorders, inherited and acquired. Most people are familiar with the bleeding disorder congenital hemophilia, but the most common inherited bleeding disorder is von Willebrand disease (VWD). Acquired bleeding disorders are more rare than congenital bleeding disorders like VWD and hemophilia, and they can develop at any age. For example, in acquired hemophilia,…

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 MASAC guidelines for bleeding disorders

The National Hemophilia Foundation’s Medical and Scientific Advisory Council (MASAC) issues expert recommendations, statements and advisories on treatment and care of bleeding disorders.

Articles of Interest

Episode 65: Inherited Bleeding Disorders

This talk goes over the most common causes of inhirted bleeding disorders. There are two major types of hemophilia – deficiency in factor VIII and IX – which predominant occur in men. Hemophilia A is deficiency of factor VIII and is the most common form of hemophilia treated with factor concentrate. Hemophilia B is less common but treatment principles are the same. There are multiple types of von Willebrand Disease as this is a complex molecular that both carries factor VIII and forms large multimers essential for platelet function. Mild forms of vWD can be treated by desmopressin but von Willebrand factor is needed for more severe types.

Bleeding Disorders in Women

The most common bleeding disorder affecting women is von Willebrand disease (VWD), which results from a deficiency or defect in the body’s ability to produce a certain protein that helps blood clot. Although VWD occurs in men and women equally, women are more likely to notice the symptoms because of heavy or abnormal bleeding during their menstrual periods and after childbirth.

EM@3AM: Bleeding Disorders

vWD is the most common inherited bleeding disorder, but do not forget to evaluate for the acquired form.

Factor First! Hemophilia management in the Emergency Department

The three most common diseases that will present to the ED are Hemophilia A, Hemophilia B, and Von Willebrands. The hemophilias are further subcategorized by severity. You should at the very least know (if you can) the severity of hemophilia or sub-type of von Willebrands. Give factor first if the patient warrants it, and come back to learn the interesting details of their disease later. These patients always need to be a triage priority as well.

Hematologic Emergencies and Life-Threatening Bleeding Disorders: Differential Diagnosis, Evaluation, and Management - Part I

The first part of this two-part series focuses on platelet-related disorders, including diseases characterized by deficient platelet production, as well as increased platelet destruction. Such conditions as drug-induced thrombocytopenia, idiopathic thrombocytopenic purpura (ITP), disseminated intravascular coagulation (DIC), and thrombotic thrombocytopenic purpura (TTP) are discussed in detail, and accompanied by specific evaluative techniques for confirming the diagnosis and management strategies that will reduce the risk of hemorrhage-related complications and improve patient outcomes.

Hematologic Emergencies and Life-Threatening Bleeding Disorders: Differential Diagnosis, Evaluation, and Management - Part II

Part I of this two-part series outlined platelet-related disorders that could lead to hemorrhagic emergencies. A number of other conditions, including hemoglobinopathies and other inherited disorders, can produce a wide range of symptoms. Transfusion reactions also must be recognized by the emergency physician and managed promptly.

Hemophilia: What’s so Bloody Funny?

Hemophilia is a set of X-linked recessive disorders of clotting factors. Deficiencies in factor VIII and factor IX produce hemophilia A and B respectively. The clinical manifestations of hemophilia A and B are practically indistinguishable and characterized primarily by easy bruising and recurrent bleeding into joints and deep muscles. Hemarthrosis is the most common complication of hemophilia with elbows, knees, and ankles most often affected. An important point to emphasize is patients may develop pain in the affected joint before any clinical signs can be detected, so it is important that even vague joint symptoms be taken seriously.

Hemophilia: When the Bleeding Won`t Stop

While the majority of hemophiliacs receive infusions of factor VIII or IX, between 15%–20% will develop an antibody called an inhibitor that will prevent the artificial clotting factor from being able to clot the blood.2 When people are diagnosed with inhibitors, treatment becomes more expensive and difficult to obtain, as more or different types of clotting factors are needed to maintain a normal lifestyle. One of the leading areas of hemophilia research involves inhibitors.

Living with a rare bleeding disorder

There are different bleeding disorders, including hemophilia, Von Willebrand disease, or a rare factor deficiency. Each of these disorders indicate a missing or defective clotting factor that makes it more difficult for an individual’s blood to clot properly. My bleeding disorder, afibrinogenemia, would fall under the rare factor deficiency category of bleeding disorders.

Management of rare acquired bleeding disorders

Autoantibodies toward clotting factors may develop in people suffering from autoimmune or neoplastic diseases, after drug intake or even in subjects without apparent conditions. They are more commonly directed against factor VIII (FVIII) or von Willebrand factor leading to acquired hemophilia A or acquired von Willebrand syndrome, respectively. Rarely, autoantibodies develop against other clotting factors, such as fibrinogen, FII, FV, FVII, FX, FXI, and FXIII.

Managing Hemophilia in the ED: All Bleeding Stops Eventually

Ideally, use the factor the patient takes at home to reduce development of inhibitors. Remember, these patients probably know their disease better than you do. If a patient does not know their own factor activity, assume that it is 0% for purposes of correction.

Treatment of Persons with Inherited Bleeding Disorders (PwIBD) in the Emergency Room

TREAT FIRST > INVESTIGATE LATER. It is recommended that treatment be given before any diagnostics studies (i.e., X-rays, CT scans, etc.) are performed to evaluate a suspected bleeding problem or injury, especially in the case of head trauma or suspected intracranial hemorrhage. Early treatment can mitigate further bleeding or complications.

Resources

BleedingDisorders.com

Whether you live with a bleeding disorder or care for someone who does, you'll find information that matters most. Just select your condition and life stage above to create your own personalized dashboard.

Bleeding Disorders

Aggressive treatment of hemophilia patients with head trauma is imperative as signs and symptoms of intracranial bleeding may be delayed. Even though initial imaging studies may be normal, factor replacement is indicated and careful monitoring of the patient is crucial to detect subtle changes in mental status.

Lone Star Bleeding Disorders Foundation

The Lone Star Bleeding Disorders Foundation is dedicated to improving the quality of life for South Texans affected by rare bleeding disorders through education, advocacy efforts to ensure access to care, supporting research for better treatments and a cure, and raising public awareness.

StatPearls

Hereditary bleeding disorders occur due to the absence or deficiency of specific clotting proteins. The three most common hereditary bleeding disorders are hemophilia A (factor VIII deficiency), hemophilia B (factor IX deficiency) and von Willebrand disease.