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© Shutterstock
0 / 30 Fotos
Talmud
- Hemophilia, a mostly inherited genetic disorder, was recognized in the ancient world as instances of excessive bleeding. The Talmud, the central text of Rabbinic Judaism, stated that baby boys did not have to be circumcised if two of their brothers had previously died from the procedure by bleeding out.
© Getty Images
1 / 30 Fotos
The Bible
- The New Testament references a woman who had hemorrhaged for 12 years before touching the hem of Jesus' garment, after which the bleeding ceased.
© Getty Images
2 / 30 Fotos
Kitab al-Tasrif
- The first medical professional to describe the disorder was a 10th-century Arab physician called Al-Zahrawi, also known as Abulcasis, or Abu Khasim. His principal work is the Kitab al-Tasrif (pictured), a 30-volume encyclopedia of medical practices. In it, he identifies the hereditary nature of hemophilia and describes families whose male relatives succumbed from uncontrolled bleeding after trauma.
© Getty Images
3 / 30 Fotos
John Conrad Otto (1774–1844)
- In 1803, Philadelphia physician John Conrad Otto published an account recognizing that a hemorrhagic bleeding disorder primarily affected men, a condition existing in certain families in which he called the affected males "bleeders." Pictured is an engraved view of the Institute of the Pennsylvania Hospital in Philadelphia, Pennsylvania, circa 1800. Ten years later, John Hay offered the idea in the New England Journal of Medicine that affected men could pass the trait for a bleeding disorder to their unaffected daughters.
© Getty Images
4 / 30 Fotos
"Haemorrhaphilia"
- The term hemophilia (the American spelling of haemophilia) is derived from "haemorrhaphilia," which was used in a description of the condition written by Friedrich Hopff in 1828 while he was a student at the University of Zurich (pictured).
© Getty Images
5 / 30 Fotos
Erik Adolf von Willebrand (1870–1949)
- Finnish doctor Erik Adolf von Willebrand published a paper in 1924 describing a hereditary bleeding disorder similar to hemophilia localized in the Åland Islands (pictured), southwest of Finland. He termed his discovery "pseudohemophilia," a bleeding disorder affecting men and women equally. It was later named von Willebrand disease.
© Getty Images
6 / 30 Fotos
Alfredo Pavlovsky (1907–1984)
- A little over 20 years later in 1947, Argentinian physician Alfredo Pavlovsky declared a major breakthrough in Buenos Aires (pictured) distinguished two types of hemophilia in his lab—A and B. Note: Hemophilia Factor I deficiency was first described in 1920. Factors II and V deficiency were identified in the 1940s.
© Getty Images
7 / 30 Fotos
"Royal disease"
- Hemophilia is often referred to as the "royal disease," a reference to the disorder featuring prominently in European royalty throughout the second half of the 19th-century and the first half of the 20th century. Queen Victoria, pictured in 1870 with her family (her husband Albert had died in 1861), is believed to have been the carrier of hemophilia B, or factor IX deficiency. She passed the mutation on to three of her nine children, including son Leopold.
© Getty Images
8 / 30 Fotos
Prince Leopold, Duke of Albany (1853–1884)
- Prince Leopold was born with hemophilia, which contributed to his death following a fall at the age of 30. The monarch's daughters, Alice and Beatrice, passed the trait on to several of their children, who married into the royal families of Spain, Germany, and Russia.
© Getty Images
9 / 30 Fotos
Tsarevich Alexei (1904-1918)
- Princess Alice's daughter, Alexandra, married Nicholas II of Russia and became Empress Alexandra Feodorovna Romanov. Their son and heir, Tsarevich Alexei, famously suffered from hemophilia, which he'd inherited from his mother. A sick child, Alexei was often bedridden.
© Getty Images
10 / 30 Fotos
Rasputin (1869–1916)
- Alexandra sought the help of a mystic, Grigori Rasputin, in finding a cure for Alexi. Rasputin's association with the Romanovs led to his rise in prominence at the imperial court. The royal family eventually met their deaths during the Russian Revolution.
© Getty Images
11 / 30 Fotos
Infant Don Gonzalo (1914–1934)
- Meanwhile the royal strain of hemophilia continued to spread. In Spain, Queen Victoria's youngest daughter, Princess Beatrice, had a daughter, Victoria Eugenie of Battenberg, who later became Queen of Spain. Two of her sons, Alfonso (1907–1938) and the younger Infante Gonzalo (pictured), were hemophiliacs. Both died from internal bleeding after each was involved in separate minor car accidents. Hemophilia was ultimately carried through various royal family members for three generations after Victoria, before abruptly disappearing.
© Getty Images
12 / 30 Fotos
Low life expectancy
- In the early 1900s, life expectancy for a hemophiliac was 13 years. Anybody surviving into their twenties was an exception. Treatments available at the time included inhaling oxygen (pictured), and the use of thyroid gland or bone marrow.
© Getty Images
13 / 30 Fotos
Snakes alive!
- A minor breakthrough in the fight against hemophilia occurred in the early 1930s when it was discovered that diluting certain snake venoms caused blood to clot.
© Getty Images
14 / 30 Fotos
Blood plasma
- In 1937, anti-hemophilia globulin was found in blood plasma. This helped decrease the clotting time in patients suffering from the disorder.
© Getty Images
15 / 30 Fotos
Transfusions
- By 1960, the average life expectancy for a person with severe hemophilia was still less than 20 years old. By now, however, fresh frozen plasma was being transfused in patients at hospitals, albeit in necessary large volumes. Pictured is a youngster being treated at a Swiss Red Cross holiday camp for hemophiliac boys in Arcegno.
© Getty Images
16 / 30 Fotos
Judith Graham Pool (1919–1975)
- The mid-1960s saw another major breakthrough when Judith Graham Pool, a researcher at Stanford University (pictured), discovered a method for the production of an antihaemophilic factor known as cryoprecipitate and rich in Factor III.
© Getty Images
17 / 30 Fotos
Cryoprecipitate
- Cryoprecipitated AHF was approved for use in 1971. Freeze-dried powdered concentrates of Cryoprecipitated AHF containing factor VIII and IX became widely available and allowed patients to "self-infuse" factor products in the comfort of their own homes (pictured).
© Getty Images
18 / 30 Fotos
Blood contamination
- Further antihaemophilic research received a major blow in the mid-1980s when it was confirmed that HIV/AIDS could be transmitted through the use of blood and blood products, including those used to treat hemophilia. The plasma used to create the products was not screened or tested, neither had most of the products been subject to any form of viral inactivation.
© Shutterstock
19 / 30 Fotos
Ryan White (1971–1990)
- One hemophiliac patient accidentally infected with the AIDS virus through a blood transfusion was Indiana schoolboy Ryan White. His legal struggle to continue studying after failing to be readmitted to school following his diagnosis made international headlines.
© Getty Images
20 / 30 Fotos
Stigmatized
- Many concerned but ill-informed parents and teachers rallied against Ryan's attendance. But after a lengthy administrative appeal process, the 13-year-old was welcomed back. Ryan is pictured after his first day back in school after being allowed to return.
© Getty Images
21 / 30 Fotos
Celebrity status
- The publicity surrounding the conflict made Ryan into a popular celebrity and enthusiastic advocate for AIDS research and public education. He's pictured with movie and TV stars Marlee Matlin, Richard Dean Anderson, and Charlie Sheen at the For the Love of Children AIDS Benefit Gala in July 1988 in Century City, California.
© Getty Images
22 / 30 Fotos
Funeral
- Ryan White confounded doctors by living far longer than the six months he was originally given after his diagnosis. But on April 8, 1990, just one month before his high school graduation, he died of the disease. Pictured is his mom, Jeanne, at her son's funeral. To her right is musician Elton John, who became a friend of the family.
© Getty Images
23 / 30 Fotos
Richard Burton (1925–1984)
- Few people realize that Richard Burton was a hemophiliac. In fact, he set up the Richard Burton Hemophilia Fund along with Elizabeth Taylor in 1964.
© Getty Images
24 / 30 Fotos
Alex Bornstein
- Another celebrity hemophiliac is Alex Borstein, the voice of Lois Griffin in 'Family Guy.' The actress is a carrier of hemophilia, as well as an active advocate for the disease.
© Getty Images
25 / 30 Fotos
Areas most affected
- This image is a representation and location of the articulations mostly affected by hemophilic arthropathy (permanent joint disease): ankle, knee, hip, elbow, and shoulder. People affected by hemophilia are prone to hemorrhages at or around these zones.
© Getty Images
26 / 30 Fotos
Tell-tale symptom
- One symptom of hemophilia is easy bruising. The slightest knock or jolt can cause a hematoma. Kids with hemophilia may have many bruises of different sizes all over their bodies.
© Shutterstock
27 / 30 Fotos
Recombinant products
- The 21st century continues to bring with it new recombinant products made without human or animal plasma derivatives. This is good news, as it lowers the possibility of risk for allergic reactions to the products or inhibitors.
© Shutterstock
28 / 30 Fotos
Gene therapy
- And advances in gene therapy may reduce symptoms those with mild or moderate hemophilia might have. Ongoing trials are testing the use of viruses as vectors, or vehicles, to deliver factor IX genes into patients' livers, correcting their hemophilia. The best results so far have been found in hemophilia B. Sources: (American Journal of Managed Care) (National Hemophilia Foundation) (PBS) (Hemophilia News Today) See also: The benefits of blood donation and the celebs who support it
© Getty Images
29 / 30 Fotos
© Shutterstock
0 / 30 Fotos
Talmud
- Hemophilia, a mostly inherited genetic disorder, was recognized in the ancient world as instances of excessive bleeding. The Talmud, the central text of Rabbinic Judaism, stated that baby boys did not have to be circumcised if two of their brothers had previously died from the procedure by bleeding out.
© Getty Images
1 / 30 Fotos
The Bible
- The New Testament references a woman who had hemorrhaged for 12 years before touching the hem of Jesus' garment, after which the bleeding ceased.
© Getty Images
2 / 30 Fotos
Kitab al-Tasrif
- The first medical professional to describe the disorder was a 10th-century Arab physician called Al-Zahrawi, also known as Abulcasis, or Abu Khasim. His principal work is the Kitab al-Tasrif (pictured), a 30-volume encyclopedia of medical practices. In it, he identifies the hereditary nature of hemophilia and describes families whose male relatives succumbed from uncontrolled bleeding after trauma.
© Getty Images
3 / 30 Fotos
John Conrad Otto (1774–1844)
- In 1803, Philadelphia physician John Conrad Otto published an account recognizing that a hemorrhagic bleeding disorder primarily affected men, a condition existing in certain families in which he called the affected males "bleeders." Pictured is an engraved view of the Institute of the Pennsylvania Hospital in Philadelphia, Pennsylvania, circa 1800. Ten years later, John Hay offered the idea in the New England Journal of Medicine that affected men could pass the trait for a bleeding disorder to their unaffected daughters.
© Getty Images
4 / 30 Fotos
"Haemorrhaphilia"
- The term hemophilia (the American spelling of haemophilia) is derived from "haemorrhaphilia," which was used in a description of the condition written by Friedrich Hopff in 1828 while he was a student at the University of Zurich (pictured).
© Getty Images
5 / 30 Fotos
Erik Adolf von Willebrand (1870–1949)
- Finnish doctor Erik Adolf von Willebrand published a paper in 1924 describing a hereditary bleeding disorder similar to hemophilia localized in the Åland Islands (pictured), southwest of Finland. He termed his discovery "pseudohemophilia," a bleeding disorder affecting men and women equally. It was later named von Willebrand disease.
© Getty Images
6 / 30 Fotos
Alfredo Pavlovsky (1907–1984)
- A little over 20 years later in 1947, Argentinian physician Alfredo Pavlovsky declared a major breakthrough in Buenos Aires (pictured) distinguished two types of hemophilia in his lab—A and B. Note: Hemophilia Factor I deficiency was first described in 1920. Factors II and V deficiency were identified in the 1940s.
© Getty Images
7 / 30 Fotos
"Royal disease"
- Hemophilia is often referred to as the "royal disease," a reference to the disorder featuring prominently in European royalty throughout the second half of the 19th-century and the first half of the 20th century. Queen Victoria, pictured in 1870 with her family (her husband Albert had died in 1861), is believed to have been the carrier of hemophilia B, or factor IX deficiency. She passed the mutation on to three of her nine children, including son Leopold.
© Getty Images
8 / 30 Fotos
Prince Leopold, Duke of Albany (1853–1884)
- Prince Leopold was born with hemophilia, which contributed to his death following a fall at the age of 30. The monarch's daughters, Alice and Beatrice, passed the trait on to several of their children, who married into the royal families of Spain, Germany, and Russia.
© Getty Images
9 / 30 Fotos
Tsarevich Alexei (1904-1918)
- Princess Alice's daughter, Alexandra, married Nicholas II of Russia and became Empress Alexandra Feodorovna Romanov. Their son and heir, Tsarevich Alexei, famously suffered from hemophilia, which he'd inherited from his mother. A sick child, Alexei was often bedridden.
© Getty Images
10 / 30 Fotos
Rasputin (1869–1916)
- Alexandra sought the help of a mystic, Grigori Rasputin, in finding a cure for Alexi. Rasputin's association with the Romanovs led to his rise in prominence at the imperial court. The royal family eventually met their deaths during the Russian Revolution.
© Getty Images
11 / 30 Fotos
Infant Don Gonzalo (1914–1934)
- Meanwhile the royal strain of hemophilia continued to spread. In Spain, Queen Victoria's youngest daughter, Princess Beatrice, had a daughter, Victoria Eugenie of Battenberg, who later became Queen of Spain. Two of her sons, Alfonso (1907–1938) and the younger Infante Gonzalo (pictured), were hemophiliacs. Both died from internal bleeding after each was involved in separate minor car accidents. Hemophilia was ultimately carried through various royal family members for three generations after Victoria, before abruptly disappearing.
© Getty Images
12 / 30 Fotos
Low life expectancy
- In the early 1900s, life expectancy for a hemophiliac was 13 years. Anybody surviving into their twenties was an exception. Treatments available at the time included inhaling oxygen (pictured), and the use of thyroid gland or bone marrow.
© Getty Images
13 / 30 Fotos
Snakes alive!
- A minor breakthrough in the fight against hemophilia occurred in the early 1930s when it was discovered that diluting certain snake venoms caused blood to clot.
© Getty Images
14 / 30 Fotos
Blood plasma
- In 1937, anti-hemophilia globulin was found in blood plasma. This helped decrease the clotting time in patients suffering from the disorder.
© Getty Images
15 / 30 Fotos
Transfusions
- By 1960, the average life expectancy for a person with severe hemophilia was still less than 20 years old. By now, however, fresh frozen plasma was being transfused in patients at hospitals, albeit in necessary large volumes. Pictured is a youngster being treated at a Swiss Red Cross holiday camp for hemophiliac boys in Arcegno.
© Getty Images
16 / 30 Fotos
Judith Graham Pool (1919–1975)
- The mid-1960s saw another major breakthrough when Judith Graham Pool, a researcher at Stanford University (pictured), discovered a method for the production of an antihaemophilic factor known as cryoprecipitate and rich in Factor III.
© Getty Images
17 / 30 Fotos
Cryoprecipitate
- Cryoprecipitated AHF was approved for use in 1971. Freeze-dried powdered concentrates of Cryoprecipitated AHF containing factor VIII and IX became widely available and allowed patients to "self-infuse" factor products in the comfort of their own homes (pictured).
© Getty Images
18 / 30 Fotos
Blood contamination
- Further antihaemophilic research received a major blow in the mid-1980s when it was confirmed that HIV/AIDS could be transmitted through the use of blood and blood products, including those used to treat hemophilia. The plasma used to create the products was not screened or tested, neither had most of the products been subject to any form of viral inactivation.
© Shutterstock
19 / 30 Fotos
Ryan White (1971–1990)
- One hemophiliac patient accidentally infected with the AIDS virus through a blood transfusion was Indiana schoolboy Ryan White. His legal struggle to continue studying after failing to be readmitted to school following his diagnosis made international headlines.
© Getty Images
20 / 30 Fotos
Stigmatized
- Many concerned but ill-informed parents and teachers rallied against Ryan's attendance. But after a lengthy administrative appeal process, the 13-year-old was welcomed back. Ryan is pictured after his first day back in school after being allowed to return.
© Getty Images
21 / 30 Fotos
Celebrity status
- The publicity surrounding the conflict made Ryan into a popular celebrity and enthusiastic advocate for AIDS research and public education. He's pictured with movie and TV stars Marlee Matlin, Richard Dean Anderson, and Charlie Sheen at the For the Love of Children AIDS Benefit Gala in July 1988 in Century City, California.
© Getty Images
22 / 30 Fotos
Funeral
- Ryan White confounded doctors by living far longer than the six months he was originally given after his diagnosis. But on April 8, 1990, just one month before his high school graduation, he died of the disease. Pictured is his mom, Jeanne, at her son's funeral. To her right is musician Elton John, who became a friend of the family.
© Getty Images
23 / 30 Fotos
Richard Burton (1925–1984)
- Few people realize that Richard Burton was a hemophiliac. In fact, he set up the Richard Burton Hemophilia Fund along with Elizabeth Taylor in 1964.
© Getty Images
24 / 30 Fotos
Alex Bornstein
- Another celebrity hemophiliac is Alex Borstein, the voice of Lois Griffin in 'Family Guy.' The actress is a carrier of hemophilia, as well as an active advocate for the disease.
© Getty Images
25 / 30 Fotos
Areas most affected
- This image is a representation and location of the articulations mostly affected by hemophilic arthropathy (permanent joint disease): ankle, knee, hip, elbow, and shoulder. People affected by hemophilia are prone to hemorrhages at or around these zones.
© Getty Images
26 / 30 Fotos
Tell-tale symptom
- One symptom of hemophilia is easy bruising. The slightest knock or jolt can cause a hematoma. Kids with hemophilia may have many bruises of different sizes all over their bodies.
© Shutterstock
27 / 30 Fotos
Recombinant products
- The 21st century continues to bring with it new recombinant products made without human or animal plasma derivatives. This is good news, as it lowers the possibility of risk for allergic reactions to the products or inhibitors.
© Shutterstock
28 / 30 Fotos
Gene therapy
- And advances in gene therapy may reduce symptoms those with mild or moderate hemophilia might have. Ongoing trials are testing the use of viruses as vectors, or vehicles, to deliver factor IX genes into patients' livers, correcting their hemophilia. The best results so far have been found in hemophilia B. Sources: (American Journal of Managed Care) (National Hemophilia Foundation) (PBS) (Hemophilia News Today) See also: The benefits of blood donation and the celebs who support it
© Getty Images
29 / 30 Fotos
The inherited royal disease: what is hemophilia?
How much do you know about this inherited bleeding disorder?
© Shutterstock
The history behind the discovery, research, and treatment of hemophilia is as complex and intriguing as the disease itself. A rare inherited genetic blood disorder that mostly affects men, hemophilia is on the rise, with more than a million men around the world stricken with the condition. But what exactly is hemophilia, and how does it impair the body's ability to produce blood clots (a process needed to stop bleeding)?
Click through and learn more about hemophilia.
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