History - Ο Αιμίλιος μας μαθαίνει για την αιμορροφιλία

A brief tour to the history of bleeding disorders

Already since Ancient times, people have recognized the association of abnormal bleeding with the male gender. In the last 200 years, science has been able to answer the biggest questions about haemophilia, continuously extending and improving the lives of people with this disorder.

2nd century AD

The Talmud, a collection of Jewish Rabbi's books, states that baby boys are not circumcised if two of their brothers have died earlier from the procedure.

10th century AD

The Arab doctor Abulcasis (or Abu Khasim) describes families whose male relatives die from uncontrolled bleeding after trauma.

1803

The American physician John Conrad Otto acknowledges that bleeding disorders primarily affect men and are common in some families. Otto calls the men who have this disorder "bleeders".

1813

The American John Hay publishes in N Eng J Med a paper in which affected men can pass the trait for a bleeding disorder to their unaffected daughters.

1828

The term "haemophilia" appears for the first time. It is thought to have been coined by German physician Johan Lucas Schönlein and his student, Friedrich Hopff.

1926

People with haemophilia are found to respond directly to blood plasma infusions when administered in a timely manner, after some spontaneous bleeding in the joints and muscles.

1937

The American doctors Arthur Patek and FHL Taylor describe blood plasma globulin, which can reduce clotting time in patients with haemophilia ("anti-haemophilic globulin").

1947

Dr. Alfredo Pavlovsky from Argentina, distinguishes haemophilia types A and B.

Δεκαετία 1950

Deficiencies of factors VII, X, XI and XII are described for the first time

1960

Factor XIII deficiency is described for the first time

1964

The British haematologist Robert Macfarlane publishes in Nature, the process of blood coagulation and the interaction of different coagulation factors (coagulation cascade).

1965

The American researcher Judith Graham Pool finds that the precipitate left after thawing of plasma is rich in factor VIII.

Δεκαετία 1970

Recombinant products of coagulation factors are being developed.

Δεκαετία 1990

• Recombinant products of coagulation factors are being developed.
• The prophylactic treatment administered 2-3 times a week in children with haemophilia is becoming a treatment of choice.
• Development of bypassing agents is an alternative therapy for patients with FVIII inhibitors, which helps to reduce bleeding and damage of the joints.

A “Royal Disease”

Haemophilia is sometimes referred to as the “royal disease” because it affected the royal families of England, Germany, Russia and Spain in the 19th and 20th centuries. Queen Victoria of England (1819-1901), is believed to have been the carrier of haemophilia B. She passed the trait on to three of her nine children. Her son Leopold died of bleeding after a fall when he was 30 years old. Her daughters, Alice and Beatrice, passed it on to several of their children. Alice’s daughter married Tsar Nicholas of Russia, and their son, Alexei had haemophilia. Haemophilia was carried through various royal family members for three generations after Victoria, then disappeared.

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Reference:

History of Bleeding Disorders, NHF. Available at https://www.hemophilia.org/Bleeding-Disorders/History-of-Bleeding-Disorders.