Hemophilia A Questions & Answers

Updated: Jan 14, 2019
  • Author: Douglass A Drelich, MD; Chief Editor: Srikanth Nagalla, MBBS, MS, FACP  more...
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Questions & Answers

Overview

What is hemophilia A?

What are the signs and symptoms of hemophilia A?

Which lab studies are performed in the evaluation of suspected hemophilia A?

Which lab values suggest hemophilia A?

What values for factor VIII (FVIII) assays indicate hemophilia A?

What is the role of imaging studies in the diagnosis of hemophilia A?

What is the role of inhibitors testing in the diagnosis of hemophilia A?

What is included in the treatment of hemophilia A?

Where is treatment for hemophilia A administered?

What are the treatment options for acute bleeds in hemophilia A?

How is the number of units of factor VIII needed to correct the factor VIII activity level in hemophilia A determined?

What are the possible regimens for factor VIII (FVIII) dosing in hemophilia A?

What types of factor VIII (FVIII) are available for the treatment of hemophilia A?

What are the attributes of desmopressin vasopressin analog for the treatment of hemophilia A?

What is the role of antifibrinolytics in the treatment of hemophilia A?

Which treatments are used for hemophilia A in patients with inhibitors of factor VIII (FVIII)?

What is hemophilia A?

What causes morbidity and death in patients with hemophilia A?

Which lab studies are performed in the evaluation of suspected hemophilia A?

What are the treatment options for hemophilia A?

How is the severity of hemophilia A classified?

What is the historical background of hemophilia A?

What is the pathophysiology of acquired hemophilia A?

What is the pathophysiology of factor VIII (FVIII) in hemophilia A?

What is the role of the von Willebrand factor (vWF) in the pathophysiology of hemophilia A?

What is the role of the coagulation system in the pathogenesis of hemophilia A?

What is the role of genetics in the pathophysiology of hemophilia A?

What is the role of factor VIII (FVIII) in the pathophysiology of hemophilia A?

What causes hemorrhage into joints in the pathophysiology of hemophilia A?

What is the role of alloantibody inhibitors in the pathogenesis of hemophilia A?

What causes inhibitor formation in patients with hemophilia A?

What are the methods used to measure levels of factor VIII (FVIII) inhibitors in hemophilia A?

What causes hemophilia A?

What is the role of factor VIII (FVIII) in the etiology of hemophilia A?

What is the role of genetics in the etiology of hemophilia A?

Which genetic mutations have a role in the etiology of hemophilia A?

What is the role of gene deletions in the etiology of hemophilia A?

What is the role of combined factor V (FV) and factor VIII (FVIII) in the etiology of hemophilia A?

What is the prevalence of hemophilia A?

What are the racial predilections of hemophilia A?

How does the presentation of hemophilia A differ in females?

In which age group is hemophilia A typically identified?

What is the prognosis of hemophilia A?

How have factor concentrates improved the prognosis of hemophilia A?

Which factors increase the risk of fractures in patients with hemophilia A?

How do viral infections affect the prognosis of hemophilia A?

What are the death rates of hemophilia A in the US?

Which infectious agents may be transmitted in factor concentrates to patients with hemophilia A?

What are possible life-threatening complications of hemophilia A?

What causes chronic debilitating joint disease in patients with hemophilia A?

What is the mortality rate for hemophilia A?

What is included in patient education for hemophilia A?

Presentation

What should be the focus of the history for suspected hemophilia A?

What are the signs and symptoms of hemorrhage in hemophilia A?

What is the evidence of infectious disease in hemophilia A?

Which clinical history is characteristic of severe hemophilia A?

Which clinical history is characteristic of mild or moderate hemophilia A?

What are the principal bleeding sites in patients with hemophilia A?

Which clinical history is characteristic of hemophilia A in the genitourinary tract?

Which clinical history is characteristic of acquired hemophilia A?

What are the systemic signs of hemorrhage in hemophilia A?

What are the physical findings characteristic of hemophilia A?

What are the signs of infectious disease in hemophilia A?

What is the manifestation of severe hemophilia A?

What are physical findings characteristic of hemophilia A after the immediate neonatal period of?

What are the physical findings characteristic of hemophilia A in growing children?

What is the manifestation of petechiae in hemophilia A?

How is hemophilia A classified?

What are pseudotumors in hemophilia A?

DDX

Which conditions should be included in the differential diagnosis of hemophilia A?

How is hemophilia A differentiated from hemophilia B?

What are the differential diagnoses for Hemophilia A?

Workup

What is the role of lab studies in the workup of hemophilia A?

What is the role of imaging studies in the workup of hemophilia A?

How is the presence of factor VIII (FVIII) inhibitor confirmed in hemophilia A?

What tests are performed for hemophilia A carrier and fetal screening?

What is included in the prenatal testing of hemophilia A carriers?

What is the role of radiography in the workup of hemophilia A?

Treatment

What are the treatment options for hemophilia A?

How are dose calculations determined in the treatment of hemophilia A?

What is the role of prophylaxis in the management of hemophilia A?

What are the treatment options for hemophilia A and inhibitors of factor VIII (FVIII)?

How is synovitis due to hemophilia A joint bleeding treated?

How can fractures be prevented in hemophilia A?

What is included in prehospital care for hemophilia A?

What should be obtained before emergency department (ED) treatment is initiated for hemophilia A?

What is included in initial emergency department (ED) care for hemophilia A?

How is minor bleeding managed in hemophilia A?

How is epistaxis or moderately severe hematuria treated in hemophilia A?

How is acute joint bleeding and expanding hematuria treated in hemophilia A?

How are life-threatening bleeding episodes initially treated in patients with hemophilia A?

What are indications for admission to an intensive care unit for treatment of hemophilia A?

When should patients with hemophilia A be transferred for treatment to a hemophilia care center?

What is included in outpatient care for hemophilia A patients with minor hemorrhage?

What is included in outpatient care of hemophilia A patients with HIV seroconversion?

Which factor VIII (FVIII) concentrates are used for the treatment of hemophilia A?

What is the indication for continuous infusion of factor VIII (FVIII) concentrates in the treatment of hemophilia A?

What is the proper dosing of factor VIII (FVIII) concentrate for the treatment of hemophilia A?

What is the role of desmopressin vasopressin analog in the treatment of hemophilia A?

How is desmopressin vasopressin analog administered for the treatment of hemophilia A?

When can desmopressin vasopressin analog be administered by intranasal spray for the treatment of hemophilia A?

What are adverse effects of desmopressin vasopressin analog for hemophilia A?

What are the most common sites of clinically significant bleeding in hemophilia A?

How are musculoskeletal bleeding episodes managed in patients with hemophilia A?

How are oral bleeding episodes managed in patients with hemophilia A?

How are GI bleeding episodes managed in patients with hemophilia A?

How are intracranial bleeding episodes managed in patients with hemophilia A?

What is the primary treatment for hemophilia A with factor VIII (FVIII) inhibitors?

What are alternative treatment options for hemophilia A with factor VIII (FVIII) inhibitors?

What is the role of emicizumab in the treatment of hemophilia A?

What is the role of recombinant activated factor VIII (FVIII) the treatment of hemophilia A?

What is the role of desensitization in the treatment of hemophilia A?

Which factors are associated with successful outcome of immune tolerance induction (ITI) in the treatment of hemophilia A?

How is immune tolerance induction (ITI) administered in the treatment of hemophilia A?

What is the role of rituximab in the treatment of hemophilia A?

What are the dosing regimen used for immune tolerance induction (ITI) in the treatment of hemophilia A?

What are the benefits of prophylactic treatment for hemophilia A?

What is the main goal of prophylactic treatment of hemophilia A?

What is the prevalence of prophylactic treatment of hemophilia A?

What is the role of recombinant FVIII products in the treatment of hemophilia A?

How is adherence to prophylaxis for hemophilia A assessed?

How is pain managed in patients with hemophilia A?

What are potential complications of hemophilia A?

How should routine vaccinations be administered to children with hemophilia A?

How is repetitive bleeding and inflammation prevented in severe hemophilia A?

What is the role of carrier testing in the prevention of hemophilia A?

What is the role of preimplantation genetic diagnosis in the prevention of hemophilia A?

Which activity restrictions are indicated for patients with hemophilia A?

What is the role of gene therapy in the treatment of hemophilia A?

What is the efficacy of gene therapy for hemophilia A?

Which specialist consultations may be beneficial in the treatment of hemophilia A?

What is the role of radiosynovectomy in the treatment of hemophilia A?

Medications

Which medications are used in the treatment of hemophilia A?

What is the calculation for number of units of factor VIII (FVIII) needed to correct factor VIII (FVIII) activity level for hemophilia A?

Where should the factor products used in the treatment of a specific patient with hemophilia A be recorded?

Which medical adjuncts to factor VIII (FVIII) are used in the treatment of hemophilia A?

Which medications in the drug class Antifibrinolytic Agents are used in the treatment of Hemophilia A?

Which medications in the drug class Vasopressin-Related are used in the treatment of Hemophilia A?

Which medications in the drug class Monoclonal Antibodies are used in the treatment of Hemophilia A?

Which medications in the drug class Coagulation Factors are used in the treatment of Hemophilia A?