Hemophilia A historical perspective

Jump to navigation Jump to search

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 historical perspective On the Web

Most recent articles

Most cited articles

Review articles

CME Programs

Powerpoint slides

Images

American Roentgen Ray Society Images of Hemophilia A historical perspective

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 historical perspective

CDC on Hemophilia A historical perspective

Hemophilia A historical perspective in the news

Blogs on Hemophilia A historical perspective

Directions to Hospitals Treating Hemophilia A

Risk calculators and risk factors for Hemophilia A historical perspective

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

Overview

Clinical distinct features of the Hemophilia disease can be noted in ancient evidences as old as 2nd century A.D. However documented scientific study of the disease was first performed in early 19th century. Hemophilia is sometimes referred to as “The Royal Disease”, as it affected many members of the English, German, Russian and Spanish monarchies in the 19th and 20th centuries.[1]Progress towards more precise and efficient recognition of the disease, its rare forms and treatments is currently on its way.

Historical Perspective

  • The disease was first described by John Conrad Otto, an American physician, in 1803 based on his observation of affected men in certain families.[2]
  • In 1947, the first distinction between Hemophilia A and Hemophilia B was made by Dr. Alfredo Pavlovsky from Argentina. He demonstrated that a blood donation from one Hemophilia patient could solve the clotting disorder of another Hemophilia patient which leads to recognition of two distinct types of factor deficiencies[3].
  • In 1965, through her work in the coagulation cascade, Dr. Judith Graham Pool discovered that cryoprecipitate left from thawing plasma was rich in Factor VIII.
  • Soon after, the first use of cryoprecipitate to treat hemophilia A was pioneered[4].

Later on, with the development of factor concentrates, the treatment approach was changed to regular intermittent factor administration rather than treating the bleeding episodes per se. With advances in orthopedic surgeries the skeletal complications of the disease were better controlled. Discovery of desmopressin in 1977 is also known as an important step towards more efficient, less harmful prevention and treatment strategy for many Hemophilia patients. Recently, more advances like introduction of recombinant coagulation factor and immune-modifications has led to more effective treatment of a wider range of Hemophilia patients[5].

References

  1. Franchini M, Mannucci PM (2012). "Past, present and future of hemophilia: a narrative review". Orphanet J Rare Dis. 7: 24. doi:10.1186/1750-1172-7-24. PMC 3502605. PMID 22551339.
  2. History of Bleeding Disorders | National Hemophilia Foundation. Available at https://www.hemophilia.org/Bleeding-Disorders/History-of-Bleeding-Disorders . Accessed on July 30, 2016
  3. PAVLOVSKY, A. (1947). CONTRIBUTION TO THE PATHOGENESIS OF HEMOPHILIA. Blood, 2(2), 185-191. Accessed July 19, 2018. Retrieved from http://www.bloodjournal.org/content/2/2/185.
  4. Pool JG, Shannon AE (1965). "Production of high-potency concentrates of antihemophilic globulin in a closed-bag system". N Engl J Med. 273 (27): 1443–7. doi:10.1056/NEJM196512302732701. PMID 5852902.
  5. Franchini M, Mannucci PM (2014). "The history of hemophilia". Semin Thromb Hemost. 40 (5): 571–6. doi:10.1055/s-0034-1381232. PMID 24911674.

Template:WH Template:WS