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During the winter of 1884, Queen Victoria’s youngest son, Prince Leopold, was convalescing in France. While there, he slipped and fell and couldn’t stop bleeding. Within a few hours, he died. Prince Leopold was the first monarch known to suffer from hemophilia—a blood disorder that causes excessive bleeding. It appears Victoria was responsible for the spread of the “royal disease” among monarchs across Europe (her relatives in Russia, Spain, France and Germany all had the disease).
There are several versions of the disease, but no one knew what type Victoria carried and this strain is now extinct. Evgeny Rogaev from the University of Massachusetts was curious about Victoria—she is particularly interesting because none of her ancestors carried the gene, and scientists believed the Queen suffered from a spontaneous mutation. Using the remains of the Romanov family (Czar Nicholas II and Czarina Alexandra’s children are Victoria’s great grandchildren), researchers examined DNA to learn more.
Publishing the results in the journal Science, Rogaev notes that Victoria and her descendants suffered from a rare and severe form of hemophilia B, or Christmas disease. Using multiplex target amplification and parallel sequencing, Rogaev and his colleagues noticed a mutation on the F9 gene on the X chromosome, which controls the blood clotting agent, Factor IX, and can alter RNA splicing. This mutation matches those found in hemophilia B. Women generally only carry the mutation on one of their X chromosomes, meaning they rarely suffer from the disease and most often pass it on to their male descendants. Those male descendants do suffer from the disease, because it expresses itself on their only X chromosome.
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That’s it? nothing about modern treatments for the disease.
I always forget my blood type (A neg?), but when I was donating regularly they often asked me to donate platelets, which involved both arms getting IVed and the blood going from 1 arm, into a machine that uses a centrifuge to separate the blood and the platelets and then puts the platelet-less blood back in the other arm.
Aside from not being able to scratch your face for 45 minutes, about twice as long as a normal donation, for fear the needles would do more damage, you often got cold, as the blood would assume the temperature of the machine and would come back in at something like room temp.
But I’m not sure exactly how they used these platelets, if there was something a hemopheliac could use regularly or just in an emergency, as well as for a surgical patients that had unusually high bleeding. And how they were administered, by IV, injection, mixed w/ plasma or platelets only.
posted by Jonny on 10-27-2009 at 4:46 pm
Prince Leopold wasn’t a monarch, he was a prince, his mother Queen Victoria was a monarch.
(captcha is Sire Gordon, hehehe!)
posted by Dianne on 10-27-2009 at 8:01 pm
Platelets are not used for hemophilia treatment; they have a different function in the clotting system. They are greatly needed, though, so good on you for taking the longer than normal time to donate them! The cold you felt was from the anticoagulant (harmless) that is added to the blood so it won’t clot before it can get back to you. In your bag of platelets, there is also some of your plasma – the way you see them when you are done is generally how they are given to a patient – through an IV. Trauma, surgery and cancer patients are among the usual recipients.
Hemophilia is a disease of clotting factors – the disease is treated by transfusing that clotting factor. I can only imagine how horrible the disease is – the slightest bump causing a painful joint bleed that won’t stop bleeding!
posted by Diane on 10-27-2009 at 8:31 pm