Hemophilia B Questions & Answers

Updated: Nov 14, 2019
  • Author: Robert A Zaiden, MD; Chief Editor: Srikanth Nagalla, MBBS, MS, FACP  more...
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Questions & Answers

Overview

What is hemophilia B?

What are the signs and symptoms of hemophilia B?

What are the signs of hemorrhage in hemophilia B?

Which lab tests are performed in the workup of hemophilia B?

Which imaging studies are performed in the workup of hemophilia B?

What is the treatment of choice for hemophilia B?

Which medications are used to treat hemophilia B?

When was hemophilia B first identified?

How is the severity of hemophilia B classified?

What is the pathophysiology of hemophilia B?

What is the role of factor IX in the pathophysiology of hemophilia B?

What is the role of the coagulation system in the pathophysiology of hemophilia B?

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

What is the pathophysiology of hemorrhage in hemophilia B?

What is the pathophysiology of inhibitor development in hemophilia B?

What causes hemophilia B?

What is the prevalence of hemophilia B?

What are the racial predilections of hemophilia B?

What are the sexual predilections of hemophilia B?

At what age do the symptoms of hemophilia B initially appear?

What is the prognosis of hemophilia B?

What is included in patient education about hemophilia B?

Presentation

Which clinical history findings are characteristic of hemophilia B?

Which physical findings are characteristic of hemophilia B?

How is hemophilia B classified?

DDX

Which conditions are included in the differential diagnoses of hemophilia B?

How is hemophilia B differentiated from hemophilia A?

What are the differential diagnoses for Hemophilia B?

Workup

What is the role of lab testing in the workup of hemophilia B?

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

When is testing for inhibitors indicated in the workup of hemophilia B?

What is the role of genetic testing in the workup of hemophilia B?

What is the role of radiography in the workups of hemophilia B?

Treatment

How is hemophilia B treated?

What is included in prehospital care for acute bleeding in patients with hemophilia B?

What is included in treatment of hemophilia B in the emergency department (ED)?

What types of factor IX concentrates are used in the treatment of hemophilia B?

What are the dosing guidelines for factor IX concentrates to treat hemophilia B?

How is hemophilia B musculoskeletal bleeding treated?

How is hemophilia B oral bleeding treated?

How is hemophilia B GI bleeding treated?

How is hemophilia B intracranial bleeding treated?

What is the role of concizumab in the treatment of hemophilia B?

How is hemophilia B treated in patients with inhibitors?

What is the role of recombinant activated FVIIa in the treatment of hemophilia B?

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

What is the role of immune tolerance induction in the treatment of hemophilia B?

What is the role of prophylaxis in the treatment of hemophilia B?

How is prophylaxis adherence assessed in patients with hemophilia B?

How is pain managed in hemophilia B?

What are the possible complications of hemophilia B?

How are bleeding episodes prevented in hemophilia B?

Which activity modifications are used in the treatment of hemophilia B?

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

Which specialist consultations are beneficial to patients with hemophilia B?

Medications

What is the role of medications in the treatment of hemophilia B?

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

Which medications in the drug class Antihemophilic Agents are used in the treatment of Hemophilia B?

Which medications in the drug class Antifibrinolytics are used in the treatment of Hemophilia B?

Which medications in the drug class Coagulation Factor VIIa (Recombinant) are used in the treatment of Hemophilia B?

Which medications in the drug class Factor IX-containing Products are used in the treatment of Hemophilia B?