von Willebrand
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Erik Adolf von Willebrand (1 February 1870 – 12 September 1949) was a Finnish physician who made major contributions to
hematology Hematology ( always spelled haematology in British English) is the branch of medicine concerned with the study of the cause, prognosis, treatment, and prevention of diseases related to blood. It involves treating diseases that affect the produc ...
. Von Willebrand disease and von Willebrand factor are named after him. He also researched metabolism, obesity and gout, and was one of the first Finnish physicians to use
insulin Insulin (, from Latin ''insula'', 'island') is a peptide hormone produced by beta cells of the pancreatic islets encoded in humans by the ''INS'' gene. It is considered to be the main anabolic hormone of the body. It regulates the metabolism o ...
to treat a
diabetic coma Diabetic coma is a life-threatening but reversible form of coma found in people with diabetes mellitus. Three different types of diabetic coma are identified: #Severe low blood sugar in a diabetic person #Diabetic ketoacidosis (usually type 1) ...
. Von Willebrand qualified in medicine in 1896 from the University of Helsinki, where he received his Ph.D. in 1899. He worked at the University of Helsinki from 1900 until 1930. From 1908 until his retirement in 1933, he was the head of the department of medicine at the Deaconess Hospital in Helsinki, where he also was physician-in-chief from 1922 to 1931. In 1924, Von Willebrand was consulted about a young girl with a bleeding disorder. He described this disorder in 1926, distinguishing it from
hemophilia Haemophilia, or hemophilia (), is a mostly inherited genetic disorder that impairs the body's ability to make blood clots, a process needed to stop bleeding. This results in people bleeding for a longer time after an injury, easy bruising, ...
. The disorder was named after him, becoming known as von Willebrand disease. The cause of the disease was later discovered to be a deficiency of a protein, now known as von Willebrand factor, that enables hemostasis.


Early life and education

Von Willebrand was born on 1 February 1870 in Vaasa, then part of the Grand Duchy of Finland in the Russian Empire. He was the third child of Fredrik Magnus von Willebrand and Signe Estlander. Fredrik had received a military education and later became an engineer. Von Willebrand belonged to a German noble family; his ancestors settled in Finland in the 18th century. His family belonged to the Swedish-speaking minority in Finland. Von Willebrand attended Vaasa Lyceum, where he excelled in botany,
chemistry Chemistry is the science, scientific study of the properties and behavior of matter. It is a natural science that covers the Chemical element, elements that make up matter to the chemical compound, compounds made of atoms, molecules and ions ...
and zoology. During this time, he spent his summers collecting botanical, lepidopterological and ornithological specimens, and his winters exploring the
Gulf of Bothnia The Gulf of Bothnia (; fi, Pohjanlahti; sv, Bottniska viken) is divided into the Bothnian Bay and Bothnian Sea, and it is the northernmost arm of the Baltic Sea, between Finland's west coast ( East Bothnia) and the Sweden's east coast (West ...
. After obtaining his baccalaureate in 1890, he began his studies at the University of Helsinki, then known as the Imperial Alexander University in Finland. Prior to qualifying as a physician in 1896, he spent the summers of 1894 and 1895 working as a junior physician at a spa in Ă…land. After graduation, Von Willebrand became assistant physician in the department of medicine at the Deaconess Hospital in Helsinki, where Ossian Schauman supervised his doctoral thesis on the changes in
hemocyte A blood cell, also called a hematopoietic cell, hemocyte, or hematocyte, is a cell produced through hematopoiesis and found mainly in the blood. Major types of blood cells include red blood cells (erythrocytes), white blood cells (leukocytes), a ...
count after
venesection In medicine, venipuncture or venepuncture is the process of obtaining intravenous access for the purpose of venous blood sampling (also called ''phlebotomy'') or intravenous therapy. In healthcare, this procedure is performed by medical labor ...
. Von Willebrand's early
hematologic Hematology ( always spelled haematology in British English) is the branch of medicine concerned with the study of the cause, prognosis, treatment, and prevention of diseases related to blood. It involves treating diseases that affect the produc ...
investigations also yielded a study on the regeneration of blood in anemia and a description of a method for the staining of blood smears using eosin and
methylene blue Methylthioninium chloride, commonly called methylene blue, is a salt used as a dye and as a medication. Methylene blue is a thiazine dye. As a medication, it is mainly used to treat methemoglobinemia by converting the ferric iron in hemoglobin ...
. He received his Ph.D. in 1899 from the University of Helsinki, for the thesis ''Zur Kenntnis der Blutveränderungen nach Aderlässen'' ("Blood Changes after Venesection").


Career

After the completion of his dissertation in 1899, Von Willebrand was appointed chief physician at a spa in
Heinola Heinola () is a town and a municipality of inhabitants () located in the eastern part of the Päijänne Tavastia region, Finland, near the borders of the South Savonia region and the Kymenlaakso region. It is the third largest municipality in ...
, and he shifted his interest from hematology to
applied physiology Applied Physiology is the study of biological systems and steps into practice. It involves the application of the knowledge of physiological properties to restore core stability and joint stability. It differs from clinical practice. See also * ...
. From 1900 to 1906, he lectured anatomy and later physiology at the University of Helsinki. During this period, he researched
thermotherapy Heat therapy, also called thermotherapy, is the use of heat in therapy, such as for pain relief and health. It can take the form of a hot cloth, hot water bottle, ultrasound, heating pad, hydrocollator packs, whirlpool baths, cordless FIR h ...
, particularly the health effects of saunas, and
phototherapy Light therapy, also called phototherapy or bright light therapy is intentional daily exposure to direct sunlight or similar-intensity artificial light in order to treat medical disorders, especially seasonal affective disorder (SAD) and circadi ...
, and invented an apparatus for measuring the dermal excretion of carbon dioxide and water. Von Willebrand's interest in internal medicine outweighed his interest in balneology and
physical therapy Physical therapy (PT), also known as physiotherapy, is one of the allied health professions. It is provided by physical therapists who promote, maintain, or restore health through physical examination, diagnosis, management, prognosis, patient ...
, however, and in 1907 he took up the position of chief physician at a municipal hospital in Helsinki. In 1908, he was appointed docent in internal medicine at the University of Helsinki. Concomitant with this appointment, he succeeded Schauman as head of the department of medicine at the Deaconess Hospital in Helsinki. He also took over the laboratory at the Deaconess Hospital, which was renowned for its hematological services. In this position, he studied metabolism, obesity and gout. In 1912, he developed a method for measuring
ketone bodies Ketone bodies are water-soluble molecules that contain the ketone groups produced from fatty acids by the liver (ketogenesis). Ketone bodies are readily transported into tissues outside the liver, where they are converted into acetyl-CoA (acetyl- ...
in urine, and the following year discussed dietetic treatments for diabetes. In 1918, nearly two decades after his last paper of the sort, Von Willebrand resumed his publishing of hematologic works, releasing studies on aplastic, hypochromic and pernicious anaemia. He also published a study regarding heart valve conditions based on data from over 10,000 autopsies performed in Helsinki from 1867 to 1916, and was a pioneer in the use of
insulin Insulin (, from Latin ''insula'', 'island') is a peptide hormone produced by beta cells of the pancreatic islets encoded in humans by the ''INS'' gene. It is considered to be the main anabolic hormone of the body. It regulates the metabolism o ...
, describing in 1922 its use in the treatment of
diabetic coma Diabetic coma is a life-threatening but reversible form of coma found in people with diabetes mellitus. Three different types of diabetic coma are identified: #Severe low blood sugar in a diabetic person #Diabetic ketoacidosis (usually type 1) ...
s. In February 1924, he successfully brought a moribund patient out of a diabetic coma through the application of insulin, using some of the first batch of the hormone ever delivered to Finland. Von Willebrand remained at the University of Helsinki until 1930. He was physician-in-chief of the Deaconess Hospital from 1922 to 1931, and became honorary professor in 1930. He remained head of the Deaconess Hospital's department of medicine until his retirement in 1933. Von Willebrand continued to publish articles after his retirement. On his 75th birthday, he released his last paper, entitled ''En genetisk blodsykdom blant innbyggerne pĂĄ Ă…land'' ("A genetic blood disease amongst the islanders of Ă…land").


Von Willebrand disease

In April 1924, Von Willebrand was consulted about Hjördis Sundblom, a five-year-old girl with a severe bleeding condition. Hjördis was the ninth of 11 children in a family from
Föglö Föglö is a group of islands and municipality in Åland, an autonomous territory of Finland. The municipality has a population of () and covers an area of of which is water. The population density is . The municipality is unilingually Swedish, ...
, one of the Åland Islands. She experienced regular bleeding from the nose, lips, gums and skin, as did six of her siblings. Three of her sisters had died due to the condition, and eight years later Hjördis died due to
menorrhagia Heavy menstrual bleeding (HMB), previously known as menorrhagia or hypermenorrhea, is a menstrual period with excessively heavy flow. It is a type of abnormal uterine bleeding Abnormal uterine bleeding (AUB), also known as (AVB) or as atypical ...
. Hjördis was brought to Von Willebrand's laboratory in Helsinki and he did not himself visit Föglö, but with the cooperation of a local schoolteacher he mapped the family pedigree. He found that the condition was present in the three previous generations, on both sides of Hjördis' family. 16 of the 35 women analysed had the condition (to a mild or severe degree), and 7 of the 31 men analysed had the condition (to a slight degree). An analysis of the heredity involved led Von Willebrand to assume the inheritance was dominant, in contrast to
hemophilia Haemophilia, or hemophilia (), is a mostly inherited genetic disorder that impairs the body's ability to make blood clots, a process needed to stop bleeding. This results in people bleeding for a longer time after an injury, easy bruising, ...
which was known to be a recessive disorder. The condition also differed from hemophilia in that it affected females at least as often as males. He published a Swedish-language article in 1926 about the disease, titled ''Hereditär pseudohemofili'' ("Hereditary pseudohemophilia"). He referenced six previous publications from the years of 1876 to 1922, totalling 19 cases on families with
bleeding diatheses In medicine (hematology), bleeding diathesis is an unusual susceptibility to bleed (hemorrhage) mostly due to hypocoagulability (a condition of irregular and slow blood clotting), in turn caused by a coagulopathy (a defect in the system of coagula ...
. The earlier authors attributed the condition to hemophilia (even in the cases of females) or to thrombopathy, which was discovered shortly before as the cause of what had previously been known as
purpura hemorrhagica Purpura haemorrhagica is a rare complication of equine strangles and is caused by bleeding from capillaries which results in red spots on the skin and mucous membranes together with oedema (swelling) of the limbs and the head. Purpura hemorrhagica ...
or Werlhof's disease. Von Willebrand also conducted hematological examinations on Hjördis and some of her family members. He recorded a normal or slightly reduced number of platelets and an undisturbed clot retraction, unlike Glanzmann's thrombasthenia. The
bleeding time Bleeding time is a medical test done on someone to assess their platelets function. It involves making a patient bleed, then timing how long it takes for them to stop bleeding using a stopwatch or other suitable devices. The term template bleedin ...
( Duke) was greatly prolonged, extending to more than 2 hours in some cases, while the
clotting time Clotting time (also called Prothrombin time) is the time required for a sample of blood to coagulate in vitro under standard conditions. There are various methods for determining the clotting time, the most common being the capillary tube method ...
was within the normal range. He concluded that the disease was either a new form of thrombopathy or a condition of the capillary endothelium. Von Willebrand published a German-language version of ''Hereditär pseudohemofili'' in 1931, which attracted international attention in the disease. Blood samples were sent to researchers at the Johns Hopkins Hospital in Baltimore, Maryland, and to several researchers in Europe, including in Leipzig. Jürgens contacted Von Willebrand and together they conducted studies on his patients. They also researched hemorheology, seeking to understand the underlying mechanism of bleeding disorders. In 1933 they co-authored an account of the disease, renaming it "constitutional thrombopathy". Numerous papers were subsequently published on the disease and it became eponymously known as von Willebrand disease between the late 1930s and the early 1940s. In 1957, it was discovered that von Willebrand disease is caused by a deficiency of a protein in blood plasma that enables hemostasis. The protein was characterised in 1971, and is known as von Willebrand factor. Von Willebrand factor has two functions. Firstly, it is the carrier molecule for factor VIII, the anti-hemophilic factor. Secondly, it promotes the aggregation of platelets and attachment to the vessel wall. In 2011, Jan van Gijn and Joost P. Gijselhart, writing in the '' Nederlands Tijdschrift voor Geneeskunde'', remarked that Von Willebrand was not far wrong when he named the disease "hereditary pseudohemophilia".


Personal life and death

In his personal life, Von Willebrand was described as a mild-mannered and modest man. He married Walborg Maria Antell in 1900, and had two daughters. As a member of the Swedish-speaking minority in Finland, he was a supporter of Ossian Schauman's ''
Folkhälsan Samfundet Folkhälsan, or in short Folkhälsan (literally "Public Health"), is a non-governmental organization based in Finland. It is active in advocating social welfare and health care for the Swedish speakers of Finland. The organization carri ...
'', which promoted social welfare and health care for Swedish-speaking Finns. His research on the bleeding condition of the Ă…land islanders was of particular interest to him, as it was a hereditary disorder that affected the Swedish-speaking minority. After his retirement in 1933 he became an avid gardener and a supporter of
nature conservation Nature conservation is the moral philosophy and conservation movement focused on protecting species from extinction, maintaining and restoring habitats, enhancing ecosystem services, and protecting biological diversity. A range of values unde ...
. Von Willebrand died on 12 September 1949, at the age of 79. In 1994, he was commemorated with a stamp issued by Ă…land. The stamp was one in a set of two: the other commemorated
Erik Jorpes Johan Erik Jorpes (born Johansson, 15 July 1894 – 10 July 1973) was a Finnish-born Swedish physician and biochemist. He identified the chemical structure of heparin and developed its clinical applications. Jorpes was the professor of medical che ...
, known for his pioneering work on
heparin Heparin, also known as unfractionated heparin (UFH), is a medication and naturally occurring glycosaminoglycan. Since heparins depend on the activity of antithrombin, they are considered anticoagulants. Specifically it is also used in the treatm ...
.


Publications

The following list of publications is compiled from Lassila, R.; Lindberg, O. (2013). "Erik von Willebrand". ''Haemophilia''. 19 (5): 645. * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * *


References


Sources

* * * {{DEFAULTSORT:Willebrand, Erik Adolf von 1870 births 1949 deaths Academic personnel of the University of Helsinki Finnish hematologists Finnish people of German descent People from Vaasa Swedish-speaking Finns University of Helsinki alumni 20th-century Finnish physicians