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{{Hemophilia A}}
{{Hemophilia A}}
{{CMG}} {{FNY}}
{{CMG}}{{AE}}{{FNY}}


==Overview==
==Overview==
*[[Hemophilia A]] presentation varies depending on the stage of the disease: <ref>Severity of Hemophilia – World Federation of Hemophilia. Available at http://www.wfh.org/en/page.aspx?pid=643. Accessed on July 30,2016 </ref>
[[Hemophilia A]] presentation varies depending on the stage of the disease.<ref>Severity of Hemophilia – World Federation of Hemophilia. Available at http://www.wfh.org/en/page.aspx?pid=643. Accessed on July 30,2016 </ref> 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]].
**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==
==Natural History==
*Clinical features are usually related to abnormal or spontaneous bleeding, and can be separated into internal bleeds and external bleeds <ref> What are the signs and symptoms of Hemophilia? – NHLBI, NIH. Available at http://www.nhlbi.nih.gov/health/health-topics/topics/hemophilia/signs. Accessed on Sept 20, 2016 </ref> <ref> Types of Bleeds | National Hemophilia Foundation. Available at https://www.hemophilia.org/Bleeding-Disorders/Types-of-Bleeds . Accessed on Sept 20, 2016 </ref>
*Clinical features are usually related to abnormal or spontaneous bleeding, and can be separated into internal bleeds and external bleeds.<ref> What are the signs and symptoms of Hemophilia? – NHLBI, NIH. Available at http://www.nhlbi.nih.gov/health/health-topics/topics/hemophilia/signs. Accessed on Sept 20, 2016 </ref><ref> Types of Bleeds | National Hemophilia Foundation. Available at https://www.hemophilia.org/Bleeding-Disorders/Types-of-Bleeds . Accessed on Sept 20, 2016 </ref>
**Internal bleeding sites include:
**Internal bleeding sites include:
***[[Hematuria]] (bleeding from kidneys or bladder)
***[[Hematuria]] (bleeding from kidneys or bladder)
Line 24: Line 21:
*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]]
*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
**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 <ref> NHF – Guardian of the Nation’s Blood Supply | National Hemophilia Foundation. Available at https://www.hemophilia.org/Bleeding-Disorders/Blood-Safety/NHF-Guardian-of-the-Nations-Blood-Supply. Accessed on Sept 20, 2016 </ref>
**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.<ref> NHF – Guardian of the Nation’s Blood Supply | National Hemophilia Foundation. Available at https://www.hemophilia.org/Bleeding-Disorders/Blood-Safety/NHF-Guardian-of-the-Nations-Blood-Supply. Accessed on Sept 20, 2016 </ref>


==Prognosis==
==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.
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. <ref> Hemophilia A | Genetic and Rare Diseases Information Center (GARD) - an NCATS Proggram. Available at https://rarediseases.info.nih.gov/diseases/6591/hemophilia-a#diseasePrognosisSection. Accessed on March 27, 2017 </ref>
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.<ref> Hemophilia A | Genetic and Rare Diseases Information Center (GARD) - an NCATS Proggram. Available at https://rarediseases.info.nih.gov/diseases/6591/hemophilia-a#diseasePrognosisSection. Accessed on March 27, 2017 </ref>


==References==
==References==

Revision as of 05:51, 16 April 2017

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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.[1] 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.[2][3]
    • Internal bleeding sites include:
    • 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.[4]

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.[5]

References

  1. ↑ Severity of Hemophilia – World Federation of Hemophilia. Available at http://www.wfh.org/en/page.aspx?pid=643. Accessed on July 30,2016
  2. ↑ What are the signs and symptoms of Hemophilia? – NHLBI, NIH. Available at http://www.nhlbi.nih.gov/health/health-topics/topics/hemophilia/signs. Accessed on Sept 20, 2016
  3. ↑ Types of Bleeds | National Hemophilia Foundation. Available at https://www.hemophilia.org/Bleeding-Disorders/Types-of-Bleeds . Accessed on Sept 20, 2016
  4. ↑ NHF – Guardian of the Nation’s Blood Supply | National Hemophilia Foundation. Available at https://www.hemophilia.org/Bleeding-Disorders/Blood-Safety/NHF-Guardian-of-the-Nations-Blood-Supply. Accessed on Sept 20, 2016
  5. ↑ Hemophilia A | Genetic and Rare Diseases Information Center (GARD) - an NCATS Proggram. Available at https://rarediseases.info.nih.gov/diseases/6591/hemophilia-a#diseasePrognosisSection. Accessed on March 27, 2017

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