Hemophilia A natural history, complications and prognosis

Hemophilia A Microchapters
Home
Patient Information
Overview
Historical Perspective
Classification
Pathophysiology
Causes
Differentiating Hemophilia A from other Diseases
Epidemiology and Demographics
Risk Factors
Screening
Natural History, Complications and Prognosis
Diagnosis
Diagnostic Study of Choice
History and Symptoms
Physical Examination
Laboratory Findings
Electrocardiogram
X Ray
CT
MRI
Echocardiography or Ultrasound
Other Imaging Findings
Other Diagnostic Studies
Treatment
Medical Therapy
Surgery
Primary Prevention
Secondary Prevention
Cost-Effectiveness of Therapy
Future or Investigational Therapies
Case Studies
Case #1
Hemophilia A natural history, complications and prognosis On the Web
Most recent articles
Most cited articles
Review articles
CME Programs
Powerpoint slides
Images
American Roentgen Ray Society Images of Hemophilia A natural history, complications and prognosis All Images X-rays Echo & Ultrasound CT Images MRI
Ongoing Trials at Clinical Trials.gov
US National Guidelines Clearinghouse
NICE Guidance
FDA on Hemophilia A natural history, complications and prognosis
CDC on Hemophilia A natural history, complications and prognosis
Hemophilia A natural history, complications and prognosis in the news
Blogs on Hemophilia A natural history, complications and prognosis
Directions to Hospitals Treating Hemophilia A
Risk calculators and risk factors for Hemophilia A natural history, complications and prognosis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Fahd Yunus, M.D. [2]

Overview

Hemophilia A presentation varies depending on the stage of the disease. People with mild hemophilia (5-40% of factor VIII activity in the blood) generally present with excessive bleeding following surgery (such as a dental procedure) or trauma. They may remain asymptomatic otherwise for long period of time, even into late adulthood. People with moderate hemophilia (1-5% of factor VIII activity in the blood) have presentation ranging between mild and severe forms. They present earlier than patients with mild hemophilia, and may bleed following minor trauma. People with severe hemophilia (less than 1% of factor VIII in blood) present sooner in life with abnormal bleeding episodes, usually in the first year of life. They are also at risk for spontaneous hemorrhages, i.e. unprovoked bleeding episodes, frequently in the joints and muscles.

Natural History

• Clinical features are usually related to abnormal or spontaneous bleeding, and can be separated into internal bleeds and external bleeds.
  • Internal bleeding sites include:
    • Hematuria (bleeding from kidneys or bladder)
    • Melena or hematochezia (bleeding into the GI tract)
    • Hemarthroses and hematomas (bleeding into joints and muscle)
    • Bleeding into the brain (potentially catastrophic)
  • External bleeding sites include:
    • Heavy bleeding from a minor cut
    • Excessive bleeding from surgical or traumatic wound
    • Epistaxis or bleeding in gums (though not as commonly seen as in platelet disorders)

Complications

• Many of the long-term sequelae of hemophilia A are either related to the morbidity of severe bleeds, or from side effects of frequent transfusions
  • Recurrent episodes of hemarthroses can cause arthritis and eventual destruction of the joint space
  • Frequent transfusions have resulted in high profile cases of hemophiliacs becoming infected with blood-borne viruses (i.e. HIV and Hepatitis C). With improved screening of blood products, the risks of these infections has been substantially reduced.

Prognosis

With appropriate assistance and education, patients with Hemophilia A can live productive lives, both in terms of longevity and quality of life. The prognosis of these patients is helped greatly with the availability of replacement therapy. Patients with hemophilia A have approximately twice the mortality of healthy male population. Patients with severe hemophilia A have a mortality rate about 4-6 times higher.