Hemophilia historical perspective: Difference between revisions

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==Overview==
==Overview==
Hemophilia is considered a very old disease with its history dating back to the 2nd century AD. The first modern descriptions of the condition appeared during the 1800s. Extensive work has been done over the centuries regarding the classification, inheritance pattern, and treatment of hemophilia.
Hemophilia is considered a very old [[disease]] with its history dating back to the 2nd century AD. The first modern descriptions of the condition appeared during the 19th century. Extensive work has been done over the centuries regarding the classification, [[Heredity|inheritance]] pattern, and treatment of hemophilia.


==Historical Perspective==
==Historical Perspective==
===Discovery===
===Discovery===
*References to a condition associated with bleeding and suggestive of hemophilia date back to the 2nd century AD.<ref>{{cite book | last = Brinkhous | first = K. M. | title = Handbook of hemophilia | publisher = Excerpta Medica Sole distributors for the U.S.A. and Canada, American Elsevier Pub. Co | location = Amsterdam New York | year = 1975 | isbn = 9789021920962 }}</ref><ref name="pmid1903310">{{cite journal |vauthors=Rosendaal FR, Smit C, Briët E |title=Hemophilia treatment in historical perspective: a review of medical and social developments |journal=Ann. Hematol. |volume=62 |issue=1 |pages=5–15 |date=February 1991 |pmid=1903310 |doi= |url=}}</ref>
*References to a condition associated with [[bleeding]] and suggestive of hemophilia date back to the 2nd century AD.<ref>{{cite book | last = Brinkhous | first = K. M. | title = Handbook of hemophilia | publisher = Excerpta Medica Sole distributors for the U.S.A. and Canada, American Elsevier Pub. Co | location = Amsterdam New York | year = 1975 | isbn = 9789021920962 }}</ref><ref name="pmid1903310">{{cite journal |vauthors=Rosendaal FR, Smit C, Briët E |title=Hemophilia treatment in historical perspective: a review of medical and social developments |journal=Ann. Hematol. |volume=62 |issue=1 |pages=5–15 |date=February 1991 |pmid=1903310 |doi= |url=}}</ref>
*Ancient religious script compilations, such as The Babylonian Tarmud, have also mentioned the condition along with relative fatal bleeding episode prevention.<ref name="pmid1903310">{{cite journal |vauthors=Rosendaal FR, Smit C, Briët E |title=Hemophilia treatment in historical perspective: a review of medical and social developments |journal=Ann. Hematol. |volume=62 |issue=1 |pages=5–15 |date=February 1991 |pmid=1903310 |doi= |url=}}</ref><ref name="Ingram1997">{{cite journal|last1=Ingram|first1=G. I. C.|title=The history of haemophilia*,†|journal=Haemophilia|volume=3|issue=S1|year=1997|pages=5–15|issn=13518216|doi=10.1111/j.1365-2516.1997.tb00168.x}}</ref>
*Ancient religious script compilations, such as The Babylonian Tarmud, have also mentioned the condition along with relative fatal [[bleeding]] episode [[Prevention (medical)|prevention]].<ref name="pmid1903310">{{cite journal |vauthors=Rosendaal FR, Smit C, Briët E |title=Hemophilia treatment in historical perspective: a review of medical and social developments |journal=Ann. Hematol. |volume=62 |issue=1 |pages=5–15 |date=February 1991 |pmid=1903310 |doi= |url=}}</ref><ref name="Ingram1997">{{cite journal|last1=Ingram|first1=G. I. C.|title=The history of haemophilia*,†|journal=Haemophilia|volume=3|issue=S1|year=1997|pages=5–15|issn=13518216|doi=10.1111/j.1365-2516.1997.tb00168.x}}</ref>
*Abu Qasim Khalaf Ibn Abbas Al Zahrawi, a pioneer of modern surgery, known in the West as Albucasis or Zahravius, described potential hemophilia cases in the 10th century.
*Abu Qasim Khalaf Ibn Abbas Al Zahrawi, a pioneer of modern [[surgery]], known in the West as Albucasis or Zahravius, described suspected hemophilia cases in the 10th century.
*G. W. Consbruch of Bielefeld, Germany, described a bleeding disease very similar to hemophilia in 1793.
*G. W. Consbruch of Bielefeld, Germany, described a [[bleeding]] [[disease]] very similar to hemophilia in 1793.
*Dr John Conrad Otto, an American physician, takes the credit for the first modern description of hemophilia in 1803. He described a bleeding disorder, transmitted via unaffected females and affecting only males. His work was published under the title “An account of an hemorrhagic disposition existing in certain families”.<ref name="pmid8653976">{{cite journal |vauthors=Otto JC |title=An account of an hemorrhagic disposition existing in certain families |journal=Clin. Orthop. Relat. Res. |volume= |issue=328 |pages=4–6 |date=July 1996 |pmid=8653976 |doi= |url=}}</ref>
*Dr John Conrad Otto, an American [[physician]], takes the credit for the first modern description of hemophilia in 1803. He described a [[bleeding]] [[Disorder (medicine)|disorder]], [[Transmission (medicine)|transmitted]] via unaffected females and affecting only males. His work was published under the title “An account of an hemorrhagic disposition existing in certain families”.<ref name="pmid8653976">{{cite journal |vauthors=Otto JC |title=An account of an hemorrhagic disposition existing in certain families |journal=Clin. Orthop. Relat. Res. |volume= |issue=328 |pages=4–6 |date=July 1996 |pmid=8653976 |doi= |url=}}</ref>
*In 1813, John F. Hay published his first analysis of a hemophilia family tree in the ''New England Journal of Medicine''.<ref name="Ingram1997">{{cite journal|last1=Ingram|first1=G. I. C.|title=The history of haemophilia*,†|journal=Haemophilia|volume=3|issue=S1|year=1997|pages=5–15|issn=13518216|doi=10.1111/j.1365-2516.1997.tb00168.x}}</ref>
*In 1813, John F. Hay published his first analysis of a hemophilia [[family tree]] in the ''[[New England Journal of Medicine]]''.<ref name="Ingram1997">{{cite journal|last1=Ingram|first1=G. I. C.|title=The history of haemophilia*,†|journal=Haemophilia|volume=3|issue=S1|year=1997|pages=5–15|issn=13518216|doi=10.1111/j.1365-2516.1997.tb00168.x}}</ref>
*Christian Friedrich Nasse, a German physician and psychiatrist, described the genetics of hemophilia in 1820 and his work resulted in Nasse's law, which states that hemophilia is transmitted entirely by unaffected females to their sons.<ref>{{cite book | last = Brinkhous | first = K. M. | title = Handbook of hemophilia | publisher = Excerpta Medica Sole distributors for the U.S.A. and Canada, American Elsevier Pub. Co | location = Amsterdam New York | year = 1975 | isbn = 9789021920962 }}</ref>
*Christian Friedrich Nasse, a German [[physician]] and [[psychiatrist]], described the [[genetics]] of hemophilia in 1820 and his work resulted in Nasse's law, which states that hemophilia is [[Transmission (medicine)|transmitted]] entirely by unaffected females to their sons.<ref>{{cite book | last = Brinkhous | first = K. M. | title = Handbook of hemophilia | publisher = Excerpta Medica Sole distributors for the U.S.A. and Canada, American Elsevier Pub. Co | location = Amsterdam New York | year = 1975 | isbn = 9789021920962 }}</ref>
*A German physician, Johann Lukas Schönlein and his student Friedrich Hopff, documented the word "hemophilia" for the first time in 1828 and the condition was described in his dissertation "About hemophilia or the hereditary predisposition to fatal bleeding".<ref>{{cite book | last = Krieger | first = Marie | title = Über die Atrophie der Menschlichen Organe bei Inanition | publisher = Springer Berlin Heidelberg Imprint Springer | location = Berlin, Heidelberg | year = 1920 | isbn = 3662229374 }}</ref>
*A German [[physician]], Johann Lukas Schönlein and his student Friedrich Hopff, documented the word "hemophilia" for the first time in 1828 and the condition was described in his dissertation with the title "About hemophilia or the hereditary predisposition to fatal bleeding".<ref>{{cite book | last = Krieger | first = Marie | title = Über die Atrophie der Menschlichen Organe bei Inanition | publisher = Springer Berlin Heidelberg Imprint Springer | location = Berlin, Heidelberg | year = 1920 | isbn = 3662229374 }}</ref>
*Nasse's law prompted further scientific debate leading to publications by J. Grandidier in 1855, John Wickham Legg in 1872, and Hermann Immermann in 1879.<ref name="Ingram1997">{{cite journal|last1=Ingram|first1=G. I. C.|title=The history of haemophilia*,†|journal=Haemophilia|volume=3|issue=S1|year=1997|pages=5–15|issn=13518216|doi=10.1111/j.1365-2516.1997.tb00168.x}}</ref>
*Nasse's law prompted further [[Science|scientific]] debate leading to [[Publication|publications]] by J. Grandidier in 1855, John Wickham Legg in 1872, and Hermann Immermann in 1879.<ref name="Ingram1997">{{cite journal|last1=Ingram|first1=G. I. C.|title=The history of haemophilia*,†|journal=Haemophilia|volume=3|issue=S1|year=1997|pages=5–15|issn=13518216|doi=10.1111/j.1365-2516.1997.tb00168.x}}</ref>
*The analysis of a hemophilia family tree by John F. Hay was followed by analyses from Sir William Osler in 1885, Kathleen P. Pratt in 1908, F. Koller and his group in 1954, and Victor A. Mckusick and Samuel I. Rapaport in 1962.
*The analysis of a hemophilia [[family tree]] by John F. Hay was followed by the analyses from Sir William Osler in 1885, Kathleen P. Pratt in 1908, F. Koller and his group in 1954, and Victor A. Mckusick and Samuel I. Rapaport in 1962.
*William Bulloch and Paul Gordon Fildes published a detailed description of the early history of hemophilia in 1912 under the title "Treasury of human inheritance".<ref>{{cite book | last = Francis, Sir | first = Bulloch, William | title = Treasury of human inheritance | publisher = Cambridge University Press | location = London | year = 1909 |}}</ref>
*William Bulloch and Paul Gordon Fildes [[Publication|published]] a detailed description of the early history of hemophilia in 1912 under the title "Treasury of human [[Heredity|inheritance]]".<ref>{{cite book | last = Francis, Sir | first = Bulloch, William | title = Treasury of human inheritance | publisher = Cambridge University Press | location = London | year = 1909 |}}</ref>


===Discovery of the Antihemophilic Globulin===
===Discovery of the Antihemophilic Globulin===
*A. E. Wright was the first who documented the prolonged clotting time of hemophilic blood in a capillary tube in 1893.<ref name="pmid20754381">{{cite journal |vauthors=Wright AE |title=On a Method of Determining the Condition of Blood Coagulability for Clinical and Experimental Purposes, and on the Effect of the Administration of Calcium Salts in Haemophilia and Actual or Threatened Haemorrhage: [Preliminary Communication] |journal=Br Med J |volume=2 |issue=1700 |pages=223–5 |date=July 1893 |pmid=20754381 |pmc=2422001 |doi= |url=}}</ref>
*A. E. Wright was the first who documented the prolonged clotting time of hemophilic [[blood]] in a capillary tube in 1893.<ref name="pmid20754381">{{cite journal |vauthors=Wright AE |title=On a Method of Determining the Condition of Blood Coagulability for Clinical and Experimental Purposes, and on the Effect of the Administration of Calcium Salts in Haemophilia and Actual or Threatened Haemorrhage: [Preliminary Communication] |journal=Br Med J |volume=2 |issue=1700 |pages=223–5 |date=July 1893 |pmid=20754381 |pmc=2422001 |doi= |url=}}</ref>
*In 1908, P. Morawitz and J. Lossen proposed a deficiency in thrombokinase associated with hemophilia and disproved the association with calcium deficiency.<ref name="pmid20754381">{{cite journal |vauthors=Wright AE |title=On a Method of Determining the Condition of Blood Coagulability for Clinical and Experimental Purposes, and on the Effect of the Administration of Calcium Salts in Haemophilia and Actual or Threatened Haemorrhage: [Preliminary Communication] |journal=Br Med J |volume=2 |issue=1700 |pages=223–5 |date=July 1893 |pmid=20754381 |pmc=2422001 |doi= |url=}}</ref>
*In 1908, P. Morawitz and J. Lossen proposed a [[deficiency]] in thrombokinase associated with hemophilia and disproved the association with [[calcium]] [[deficiency]].<ref name="pmid20754381">{{cite journal |vauthors=Wright AE |title=On a Method of Determining the Condition of Blood Coagulability for Clinical and Experimental Purposes, and on the Effect of the Administration of Calcium Salts in Haemophilia and Actual or Threatened Haemorrhage: [Preliminary Communication] |journal=Br Med J |volume=2 |issue=1700 |pages=223–5 |date=July 1893 |pmid=20754381 |pmc=2422001 |doi= |url=}}</ref>
*In 1911, T. Addis investigated several blood and tissue factors and concluded that the hemophilic blood has defective prothrombin.<ref name="Addis1911">{{cite journal|last1=Addis|first1=T.|title=The pathogenesis of hereditary hæmophilia|journal=The Journal of Pathology and Bacteriology|volume=15|issue=4|year=1911|pages=427–452|issn=0368-3494|doi=10.1002/path.1700150402}}</ref>
*In 1911, T. Addis investigated several [[blood]] and [[Tissue factor|tissue factors]] and concluded that the hemophilic [[blood]] has defective [[prothrombin]].<ref name="Addis1911">{{cite journal|last1=Addis|first1=T.|title=The pathogenesis of hereditary hæmophilia|journal=The Journal of Pathology and Bacteriology|volume=15|issue=4|year=1911|pages=427–452|issn=0368-3494|doi=10.1002/path.1700150402}}</ref>
*In 1931, P. Govaerts and A. Gatia proposed that the platelets from hemophilic blood behaved normally when shifted to normal plasma. This finding hinted towards a deficiency in the plasma.<ref name="Ingram1997">{{cite journal|last1=Ingram|first1=G. I. C.|title=The history of haemophilia*,†|journal=Haemophilia|volume=3|issue=S1|year=1997|pages=5–15|issn=13518216|doi=10.1111/j.1365-2516.1997.tb00168.x}}</ref>
*In 1931, P. Govaerts and A. Gatia proposed that the [[Platelet|platelets]] from hemophilic [[blood]] behaved normally when shifted to normal [[Blood plasma|plasma]]. This finding hinted towards a [[deficiency]] in the [[Blood plasma|plasma]].<ref name="Ingram1997">{{cite journal|last1=Ingram|first1=G. I. C.|title=The history of haemophilia*,†|journal=Haemophilia|volume=3|issue=S1|year=1997|pages=5–15|issn=13518216|doi=10.1111/j.1365-2516.1997.tb00168.x}}</ref>
*In 1934, S. Van Creveld demonstrated that a “dispersed protein” fraction obtained from the serum decreased the clotting time of hemophilic blood.<ref name="CreveldJordan2009">{{cite journal|last1=Creveld|first1=S.|last2=Jordan|first2=F. L. J.|last3=Punt|first3=K.|title=Deficiency ot Anti-Hemophilic Factor in a Woman, Combined with a Disturbance in Vascular Function.1|journal=Acta Medica Scandinavica|volume=151|issue=5|year=2009|pages=381–389|issn=00016101|doi=10.1111/j.0954-6820.1955.tb10306.x}}</ref>
*In 1934, S. Van Creveld demonstrated that a “dispersed [[protein]]” fraction obtained from the [[serum]] decreased the clotting time of hemophilic [[blood]].<ref name="CreveldJordan2009">{{cite journal|last1=Creveld|first1=S.|last2=Jordan|first2=F. L. J.|last3=Punt|first3=K.|title=Deficiency ot Anti-Hemophilic Factor in a Woman, Combined with a Disturbance in Vascular Function.1|journal=Acta Medica Scandinavica|volume=151|issue=5|year=2009|pages=381–389|issn=00016101|doi=10.1111/j.0954-6820.1955.tb10306.x}}</ref>
*In 1936, A.J. Patek and F.H.L. Taylor proposed in their publication in ''Science'' that in normal blood and in citrated normal plasma rendered free from platelets by Berkefeld filtration, a substance was identified which, in small quantities, reduced the clotting time of hemophilic blood. Between 1936 and 1946, this research group published multiple papers supporting their original hypothesis<ref>{{cite journal|title=Commentary on and reprint of Patek AJ Jr, Taylor FHL, Hemophilia. II. Some properties of substances obtained from human plasma effective in accelerating coagulation of hemophiliac blood, in Journal of Clinical Investigation (1937) 16:113–124|year=2000|pages=573–585|doi=10.1016/B978-012448510-5.50144-8}}</ref><ref name="pmid4887314">{{cite journal |vauthors=Hynes HE, Owen CA, Bowie EJ, Thompson JH |title=Development of the present concept of hemophilia |journal=Mayo Clin. Proc. |volume=44 |issue=3 |pages=193–206 |date=March 1969 |pmid=4887314 |doi= |url=}}</ref>
*In 1936, A.J. Patek and F.H.L. Taylor proposed in their [[publication]] in ''[[Science (journal)|Science]]'' that in normal [[blood]] and in [[Citrate|citrated]] normal [[Blood plasma|plasma]] rendered free from [[Platelet|platelets]] by Berkefeld [[filtration]], a [[substance]] was identified which, in small quantities, reduced the clotting time of hemophilic [[blood]]. Between 1936 and 1946, this research group [[Publication|published]] multiple papers supporting their original [[hypothesis]]<ref>{{cite journal|title=Commentary on and reprint of Patek AJ Jr, Taylor FHL, Hemophilia. II. Some properties of substances obtained from human plasma effective in accelerating coagulation of hemophiliac blood, in Journal of Clinical Investigation (1937) 16:113–124|year=2000|pages=573–585|doi=10.1016/B978-012448510-5.50144-8}}</ref><ref name="pmid4887314">{{cite journal |vauthors=Hynes HE, Owen CA, Bowie EJ, Thompson JH |title=Development of the present concept of hemophilia |journal=Mayo Clin. Proc. |volume=44 |issue=3 |pages=193–206 |date=March 1969 |pmid=4887314 |doi= |url=}}</ref>
*A.J. Quick, M. Stanley-Brown, F.W. Bancroft solved the question of whether prothrombin or one of its derivatives is the deficient factor in hemophilia. They concluded that the hemophilic blood has a normal prothrombin content.<ref name="pmid6999657">{{cite journal |vauthors=Pisciotta AV |title=Concepts of haemostasis and thrombosis: A study of the coagulation defect in hemophilia and in jaundice (Quick, Stanley-Brown and Bancroft 1935). Armand J. Quick (1894-1978)--a short biography |journal=Thromb. Haemost. |volume=44 |issue=1 |pages=1–5 |date=August 1980 |pmid=6999657 |doi= |url=}}</ref>
*A.J. Quick, M. Stanley-Brown, F.W. Bancroft solved the question of whether [[Thrombin|prothrombin]] or one of its derivatives is the [[Deficiency|deficient]] factor in hemophilia. They concluded that the hemophilic [[blood]] has a normal [[Thrombin|prothrombin]] content.<ref name="pmid6999657">{{cite journal |vauthors=Pisciotta AV |title=Concepts of haemostasis and thrombosis: A study of the coagulation defect in hemophilia and in jaundice (Quick, Stanley-Brown and Bancroft 1935). Armand J. Quick (1894-1978)--a short biography |journal=Thromb. Haemost. |volume=44 |issue=1 |pages=1–5 |date=August 1980 |pmid=6999657 |doi= |url=}}</ref>
*In 1939, Brinkhous et al. confirmed A.J. Quick's findings and showed that the hemophilic blood has a delayed prothrombin conversion rate.<ref name="Brinkhous1947">{{cite journal|last1=Brinkhous|first1=K. M.|title=Clotting Defect in Hemophilia: Deficiency in a Plasma Factor Required for Platelet Utilization|journal=Experimental Biology and Medicine|volume=66|issue=1|year=1947|pages=117–120|issn=1535-3702|doi=10.3181/00379727-66-16003}}</ref>
*In 1939, Brinkhous et al. confirmed A.J. Quick's findings and showed that the hemophilic [[blood]] has a delayed [[Thrombin|prothrombin]] conversion rate.<ref name="Brinkhous1947">{{cite journal|last1=Brinkhous|first1=K. M.|title=Clotting Defect in Hemophilia: Deficiency in a Plasma Factor Required for Platelet Utilization|journal=Experimental Biology and Medicine|volume=66|issue=1|year=1947|pages=117–120|issn=1535-3702|doi=10.3181/00379727-66-16003}}</ref>
*In 1947, A.j. Quick and K.M. Brinkhous independently demonstrated that the antihemophilic globulin and platelets react together in a fashion to generate thromboplastin. They also proposed that a deficiency in antihemophilic globulin caused defective coagulation due to defects in the generation of thromboplastin.<ref name="pmid20263163">{{cite journal |vauthors=QUICK AJ |title=Studies on the enigma of the hemostatic dysfunction of hemophilia |journal=Am. J. Med. Sci. |volume=214 |issue=3 |pages=272–80 |date=September 1947 |pmid=20263163 |doi= |url=}}</ref><ref name="Brinkhous1947">{{cite journal|last1=Brinkhous|first1=K. M.|title=Clotting Defect in Hemophilia: Deficiency in a Plasma Factor Required for Platelet Utilization|journal=Experimental Biology and Medicine|volume=66|issue=1|year=1947|pages=117–120|issn=1535-3702|doi=10.3181/00379727-66-16003}}</ref>
*In 1947, A.j. Quick and K.M. Brinkhous independently demonstrated that the antihemophilic globulin and [[Platelet|platelets]] react together in a fashion to generate [[Tissue factor|thromboplastin]]. They also proposed that a [[deficiency]] in antihemophilic globulin caused defective [[coagulation]] due to defects in the generation of [[Tissue factor|thromboplastin]].<ref name="pmid20263163">{{cite journal |vauthors=QUICK AJ |title=Studies on the enigma of the hemostatic dysfunction of hemophilia |journal=Am. J. Med. Sci. |volume=214 |issue=3 |pages=272–80 |date=September 1947 |pmid=20263163 |doi= |url=}}</ref><ref name="Brinkhous1947">{{cite journal|last1=Brinkhous|first1=K. M.|title=Clotting Defect in Hemophilia: Deficiency in a Plasma Factor Required for Platelet Utilization|journal=Experimental Biology and Medicine|volume=66|issue=1|year=1947|pages=117–120|issn=1535-3702|doi=10.3181/00379727-66-16003}}</ref>


==Landmark Events in the Development of Treatment Strategies==
==Landmark Events in the Development of Treatment Strategies==
*
===Transfusion Medicine===
*In 1832, J.L. Schönlein proposed the use of [[blood transfusion]].
*In 1840, Samuel Armstrong Lane treated a case of severe postoperative [[bleeding]] by [[blood transfusion]].<ref name="Lane1840">{{cite journal|last1=Lane|first1=Samuel|title=HÆMORRHAGIC DIATHESIS.|journal=The Lancet|volume=35|issue=896|year=1840|pages=185–188|issn=01406736|doi=10.1016/S0140-6736(00)40031-0}}</ref>
*A German [[surgeon]], Ernst von Bergmann, proposed the idea of using modified [[Saline (medicine)|saline solution]] as an alternative to [[blood transfusion]].<ref>{{cite book | last = Bergmann | first = E. v. | title = Die Schicksale der Transfusion im Letzten Decennium : Rede, Gehalten zur Feier des Stiftungstages der Militärärztlichen Bildungsanstalten am 2. August 1883 | publisher = Springer Berlin Heidelberg | location = Berlin | year = 2013 | isbn = 9783642619298 }}</ref>
*In 1879, H. Kronecker and J. Sander introduced the administration of [[Saline (medicine)|saline]] and it was subsequently improved by Sydney Ringer with the addition of electrolytes.<ref name="Ringer1882">{{cite journal|last1=Ringer|first1=Sydney|title=Regarding the Action of Hydrate of Soda, Hydrate of Ammonia, and Hydrate of Potash on the Ventricle of the Frog's Heart|journal=The Journal of Physiology|volume=3|issue=3-4|year=1882|pages=195–202|issn=00223751|doi=10.1113/jphysiol.1882.sp000095}}</ref>
*In 1930, Karl Landsteiner won the Nobel Prize for his discovery of the [[Human blood group systems|human blood groups]] published under the title "The [[agglutination]] phenomenon of normal human [[blood]]".<ref name="TanGraham2013">{{cite journal|last1=Tan|first1=SY|last2=Graham|first2=C|title=Karl Landsteiner (1868–1943): Originator of ABO blood classification|journal=Singapore Medical Journal|volume=54|issue=5|year=2013|pages=243–244|issn=00375675|doi=10.11622/smedj.2013099}}</ref><ref>{{cite journal|title=Commentary on and reprint of Landsteiner K, Ueber Agglutinationserscheinungen normalen menschlichen Blute [On the agglutination of normal human blood], in Wiener Klinische Wochenschrift (1901) 14:1132–1134|year=2000|pages=769–775|doi=10.1016/B978-012448510-5.50165-5}}</ref>
*W. Schulz applied the findings of Karl Landsteiner. He cross-matched [[blood]] before he transfused it and noted that [[agglutination]] and subsequent [[Blood transfusion|transfusion]] resulted in a severe [[Collapse (medical)|collapse]], while a negative [[cross-matching]] without [[agglutination]] did not have the same result.<ref>{{cite book | last = Eckhardt | first = Christian | title = Transfusionsmedizin : Grundlagen · Therapie · Methodik | publisher = Springer Berlin Heidelberg Imprint Springer | location = Berlin, Heidelberg | year = 1988 | isbn = 9783662106020 }}</ref>
*Reuben Ottenberg and David J. Kaliski improved the [[cross-matching]] of W. Schulz to avoid [[Blood transfusion|transfusion]]-related [[Adverse effect (medicine)|adverse reactions]] and proposed the major and minor test.<ref name="OttenbergKaliski2009">{{cite journal|last1=Ottenberg|first1=Reuben|last2=Kaliski|first2=David|title=Die Gefahren der Transfusionen und deren Verhütung|journal=DMW - Deutsche Medizinische Wochenschrift|volume=39|issue=46|year=2009|pages=2243–2247|issn=0012-0472|doi=10.1055/s-0028-1128886}}</ref>
*In 1916, Thomas Addis reported that the [[coagulation]] time of hemophilic [[blood]] reduced after the [[Intravenous therapy|intravenous infusion]] of fresh human [[serum]].<ref name="Addis1916">{{cite journal|last1=Addis|first1=T.|title=The effect of intravenous injections of fresh human serum and of phosphated blood, on the coagulation time of the blood in hereditary hemophila|journal=Experimental Biology and Medicine|volume=14|issue=1|year=1916|pages=19–23|issn=1535-3702|doi=10.3181/00379727-14-14}}</ref>
*In 1935, W.M. Bendien and S. Van Creveld [[Publication|published]] their work on the isolation and [[Intravenous therapy|intravenous]] or [[Intramuscular injection|intramuscular]] administration of a “[[coagulation]]-promoting” substance which in 1934 they demonstrated as a “dispersed [[protein]]” fraction obtained from [[serum]]. They proposed that this “[[coagulation]]-promoting” substance, which was low on [[protein]], had the ability to decrease the [[coagulation]] time of hemophilic [[blood]] to within normal values.<ref name="CreveldJordan2009">{{cite journal|last1=Creveld|first1=S.|last2=Jordan|first2=F. L. J.|last3=Punt|first3=K.|title=Deficiency ot Anti-Hemophilic Factor in a Woman, Combined with a Disturbance in Vascular Function.1|journal=Acta Medica Scandinavica|volume=151|issue=5|year=2009|pages=381–389|issn=00016101|doi=10.1111/j.0954-6820.1955.tb10306.x}}</ref><ref name="Bendien1937">{{cite journal|last1=Bendien|first1=W. M.|title=INVESTIGATIONS ON HEMOPHILIA|journal=Archives of Pediatrics & Adolescent Medicine|volume=54|issue=4|year=1937|pages=713|issn=1072-4710|doi=10.1001/archpedi.1937.01980040017002}}</ref>
 
===Evolution of the Treatment Strategies===
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| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" |1840
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*First successful [[Blood transfusion|transfusion of whole blood]]<ref name="Lane1840">{{cite journal|last1=Lane|first1=Samuel|title=HÆMORRHAGIC DIATHESIS.|journal=The Lancet|volume=35|issue=896|year=1840|pages=185–188|issn=01406736|doi=10.1016/S0140-6736(00)40031-0}}</ref>
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| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" align="center" |1911
| style="padding: 5px 5px; background: #F5F5F5;" |
*Identification of [[globulin]] fraction from [[Blood plasma|plasma]] which reduced the [[coagulation]] time of hemophilic [[blood]]<ref name="Addis1911">{{cite journal|last1=Addis|first1=T.|title=The pathogenesis of hereditary hæmophilia|journal=The Journal of Pathology and Bacteriology|volume=15|issue=4|year=1911|pages=427–452|issn=0368-3494|doi=10.1002/path.1700150402}}</ref>
 
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" |1916
| style="padding: 5px 5px; background: #F5F5F5;" |
*[[Intravenous therapy|Intravenous infusion]] of fresh human [[serum]] reduced the [[coagulation]] time of hemophilic [[blood]]<ref name="Addis1916">{{cite journal|last1=Addis|first1=T.|title=The effect of intravenous injections of fresh human serum and of phosphated blood, on the coagulation time of the blood in hereditary hemophila|journal=Experimental Biology and Medicine|volume=14|issue=1|year=1916|pages=19–23|issn=1535-3702|doi=10.3181/00379727-14-14}}</ref>
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" |1934
| style="padding: 5px 5px; background: #F5F5F5;" |
*Russell’s viper [[venom]] (“Stypen”) was first used for the local control of [[bleeding]] in [[Patient|patients]] with [[hemophilia A]], [[bleeding diathesis]], and in healthy controls<ref name="MacfarlaneBarnett1934">{{cite journal|last1=Macfarlane|first1=R.G.|last2=Barnett|first2=Burgess|title=THE HÆMOSTATIC POSSIBILITIES OF SNAKE-VENOM|journal=The Lancet|volume=224|issue=5801|year=1934|pages=985–987|issn=01406736|doi=10.1016/S0140-6736(00)43846-8}}</ref>
 
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" |1935
| style="padding: 5px 5px; background: #F5F5F5;" |
*A “[[coagulation]]-promoting” substance isolated from normal [[Blood plasma|plasma]] reduced the [[coagulation]] time of hemophilic [[blood]] to within normal values when administered [[Intravenous therapy|intravenously]] or [[Intramuscular injection|intramuscularly]]<ref name="CreveldJordan2009">{{cite journal|last1=Creveld|first1=S.|last2=Jordan|first2=F. L. J.|last3=Punt|first3=K.|title=Deficiency ot Anti-Hemophilic Factor in a Woman, Combined with a Disturbance in Vascular Function.1|journal=Acta Medica Scandinavica|volume=151|issue=5|year=2009|pages=381–389|issn=00016101|doi=10.1111/j.0954-6820.1955.tb10306.x}}</ref><ref name="Bendien1937">{{cite journal|last1=Bendien|first1=W. M.|title=INVESTIGATIONS ON HEMOPHILIA|journal=Archives of Pediatrics & Adolescent Medicine|volume=54|issue=4|year=1937|pages=713|issn=1072-4710|doi=10.1001/archpedi.1937.01980040017002}}</ref>
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" |1936
| style="padding: 5px 5px; background: #F5F5F5;" |
*First evidence of a [[Precipitation (chemistry)|precipitate]] of whole [[blood plasma]] to correct [[bleeding]] time of hemophilic [[blood]]<ref>{{cite journal|title=Commentary on and reprint of Patek AJ Jr, Taylor FHL, Hemophilia. II. Some properties of substances obtained from human plasma effective in accelerating coagulation of hemophiliac blood, in Journal of Clinical Investigation (1937) 16:113–124|year=2000|pages=573–585|doi=10.1016/B978-012448510-5.50144-8}}</ref>
 
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" |1946
| style="padding: 5px 5px; background: #F5F5F5;" |
*"Antihemophilic globulin" introduced as a term<ref name="pmid16695264">{{cite journal |vauthors=Minot GR, Davidson CS, Lewis JH, Tagnon HJ, Taylor FH |title=THE COAGULATION DEFECT IN HEMOPHILIA: THE EFFECT, IN HEMOPHILIA, OF THE PARENTERAL ADMINISTRATION OF A FRACTION OF THE PLASMA GLOBULINS RICH IN FIBRINOGEN |journal=J. Clin. Invest. |volume=24 |issue=5 |pages=704–7 |date=September 1945 |pmid=16695264 |pmc=435506 |doi=10.1172/JCI101654 |url=}}</ref><ref name="pmid16695263">{{cite journal |vauthors=Taylor FH, Davidson CS, Tagnon HJ, Adams MA, Macdonald AH, Minot GR |title=STUDIES IN BLOOD COAGULATION: THE COAGULATION PROPERTIES OF CERTAIN GLOBULIN FRACTIONS OF NORMAL HUMAN PLASMA IN VITRO |journal=J. Clin. Invest. |volume=24 |issue=5 |pages=698–703 |date=September 1945 |pmid=16695263 |pmc=435505 |doi=10.1172/JCI101653 |url=}}</ref>
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" |1953
| style="padding: 5px 5px; background: #F5F5F5;" |
*First [[Prothrombin complex concentrate|prothrombin complex concentrate (ACC 76®)]] is marketed by Behringwerke AG
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" |1958
| style="padding: 5px 5px; background: #F5F5F5;" |
*[[Hemophilia A]] prophylaxis begins in Sweden<ref name="NilssonBerntorp1992">{{cite journal|last1=Nilsson|first1=I. M.|last2=Berntorp|first2=E.|last3=Löfqvist|first3=T.|last4=Pettersson|first4=H.|title=Twenty-five years' experience of prophylactic treatment in severe haemophilia A and B|journal=Journal of Internal Medicine|volume=232|issue=1|year=1992|pages=25–32|issn=09546820|doi=10.1111/j.1365-2796.1992.tb00546.x}}</ref>
 
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" |1965
| style="padding: 5px 5px; background: #F5F5F5;" |
*The revolution in the treatment of hemophilia with [[cryoprecipitate]] <ref name="PoolShannon1965">{{cite journal|last1=Pool|first1=Judith Graham|last2=Shannon|first2=Angela E.|title=Production of High-Potency Concentrates of Antihemophilic Globulin in a Closed-Bag System|journal=New England Journal of Medicine|volume=273|issue=27|year=1965|pages=1443–1447|issn=0028-4793|doi=10.1056/NEJM196512302732701}}</ref>
 
|}
===Clotting Factor Concentrates and its Evolution to Modern Treatment===
{| style="border: 0px; font-size: 90%; margin: 3px; width:650px"
| valign="top" |
|+
! style="background: #4479BA; width: 150px;" | {{fontcolor|#FFF|Year}}
! style="background: #4479BA; width: 370px;" | {{fontcolor|#FFF|Therapy}}
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" |1981
| style="padding: 5px 5px; background: #F5F5F5;" |
*First [[Pasteurization|pasteurized]] [[factor VIII]] concentrate (Haemate® P) became available in Germany
|-
| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" align="center" |1990
| style="padding: 5px 5px; background: #F5F5F5;" |
*A highly purified [[Pasteurization|pasteurized]] [[factor VIII]] concentrate Beriate® P registered in Germany
 
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" |1992
| style="padding: 5px 5px; background: #F5F5F5;" |
*The first recombinant [[factor VIII]] product is introduced and registered
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" |2004 - current
| style="padding: 5px 5px; background: #F5F5F5;" |
*Developmental breakthroughs in the recombinant [[factor VIII]] products
 
|}
==Famous Cases==
Hemophilia has been called "the [[disease]] of the kings" or "the royal [[disease]]" as several members of the European royal family have been affected by it.<ref name="Ingram1976">{{cite journal|last1=Ingram|first1=G I|title=The history of haemophilia.|journal=Journal of Clinical Pathology|volume=29|issue=6|year=1976|pages=469–479|issn=0021-9746|doi=10.1136/jcp.29.6.469}}</ref>
The following are a few famous cases of hemophilia:
*The Queen of England, Queen Victoria (1837–1901), was a carrier of hemophilia and she passed it onto her son, Leopold, who died of a [[Intracranial hemorrhage|brain hemorrhage]] when he was 31.<ref name="Ingram1976">{{cite journal|last1=Ingram|first1=G I|title=The history of haemophilia.|journal=Journal of Clinical Pathology|volume=29|issue=6|year=1976|pages=469–479|issn=0021-9746|doi=10.1136/jcp.29.6.469}}</ref>
*The [[disease]] spread to other royal families in Germany, Russia and Spain through Queen Victoria’s two daughters.
*The best known case of "the royal [[disease]]" was Tsarevich Alexei, son of the Russian Czar Nicholas II.


==References==
==References==

Latest revision as of 20:42, 24 October 2019

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Sabawoon Mirwais, M.B.B.S, M.D.[2]

Overview

Hemophilia is considered a very old disease with its history dating back to the 2nd century AD. The first modern descriptions of the condition appeared during the 19th century. Extensive work has been done over the centuries regarding the classification, inheritance pattern, and treatment of hemophilia.

Historical Perspective

Discovery

  • References to a condition associated with bleeding and suggestive of hemophilia date back to the 2nd century AD.[1][2]
  • Ancient religious script compilations, such as The Babylonian Tarmud, have also mentioned the condition along with relative fatal bleeding episode prevention.[2][3]
  • Abu Qasim Khalaf Ibn Abbas Al Zahrawi, a pioneer of modern surgery, known in the West as Albucasis or Zahravius, described suspected hemophilia cases in the 10th century.
  • G. W. Consbruch of Bielefeld, Germany, described a bleeding disease very similar to hemophilia in 1793.
  • Dr John Conrad Otto, an American physician, takes the credit for the first modern description of hemophilia in 1803. He described a bleeding disorder, transmitted via unaffected females and affecting only males. His work was published under the title “An account of an hemorrhagic disposition existing in certain families”.[4]
  • In 1813, John F. Hay published his first analysis of a hemophilia family tree in the New England Journal of Medicine.[3]
  • Christian Friedrich Nasse, a German physician and psychiatrist, described the genetics of hemophilia in 1820 and his work resulted in Nasse's law, which states that hemophilia is transmitted entirely by unaffected females to their sons.[5]
  • A German physician, Johann Lukas Schönlein and his student Friedrich Hopff, documented the word "hemophilia" for the first time in 1828 and the condition was described in his dissertation with the title "About hemophilia or the hereditary predisposition to fatal bleeding".[6]
  • Nasse's law prompted further scientific debate leading to publications by J. Grandidier in 1855, John Wickham Legg in 1872, and Hermann Immermann in 1879.[3]
  • The analysis of a hemophilia family tree by John F. Hay was followed by the analyses from Sir William Osler in 1885, Kathleen P. Pratt in 1908, F. Koller and his group in 1954, and Victor A. Mckusick and Samuel I. Rapaport in 1962.
  • William Bulloch and Paul Gordon Fildes published a detailed description of the early history of hemophilia in 1912 under the title "Treasury of human inheritance".[7]

Discovery of the Antihemophilic Globulin

  • A. E. Wright was the first who documented the prolonged clotting time of hemophilic blood in a capillary tube in 1893.[8]
  • In 1908, P. Morawitz and J. Lossen proposed a deficiency in thrombokinase associated with hemophilia and disproved the association with calcium deficiency.[8]
  • In 1911, T. Addis investigated several blood and tissue factors and concluded that the hemophilic blood has defective prothrombin.[9]
  • In 1931, P. Govaerts and A. Gatia proposed that the platelets from hemophilic blood behaved normally when shifted to normal plasma. This finding hinted towards a deficiency in the plasma.[3]
  • In 1934, S. Van Creveld demonstrated that a “dispersed protein” fraction obtained from the serum decreased the clotting time of hemophilic blood.[10]
  • In 1936, A.J. Patek and F.H.L. Taylor proposed in their publication in Science that in normal blood and in citrated normal plasma rendered free from platelets by Berkefeld filtration, a substance was identified which, in small quantities, reduced the clotting time of hemophilic blood. Between 1936 and 1946, this research group published multiple papers supporting their original hypothesis[11][12]
  • A.J. Quick, M. Stanley-Brown, F.W. Bancroft solved the question of whether prothrombin or one of its derivatives is the deficient factor in hemophilia. They concluded that the hemophilic blood has a normal prothrombin content.[13]
  • In 1939, Brinkhous et al. confirmed A.J. Quick's findings and showed that the hemophilic blood has a delayed prothrombin conversion rate.[14]
  • In 1947, A.j. Quick and K.M. Brinkhous independently demonstrated that the antihemophilic globulin and platelets react together in a fashion to generate thromboplastin. They also proposed that a deficiency in antihemophilic globulin caused defective coagulation due to defects in the generation of thromboplastin.[15][14]

Landmark Events in the Development of Treatment Strategies

Transfusion Medicine

Evolution of the Treatment Strategies
Year Therapy
1840
1911
1916
1934
1935
1936
1946
  • "Antihemophilic globulin" introduced as a term[27][28]
1953
1958
1965

Clotting Factor Concentrates and its Evolution to Modern Treatment
Year Therapy
1981
1990
1992
  • The first recombinant factor VIII product is introduced and registered
2004 - current
  • Developmental breakthroughs in the recombinant factor VIII products

Famous Cases

Hemophilia has been called "the disease of the kings" or "the royal disease" as several members of the European royal family have been affected by it.[31] The following are a few famous cases of hemophilia:

  • The Queen of England, Queen Victoria (1837–1901), was a carrier of hemophilia and she passed it onto her son, Leopold, who died of a brain hemorrhage when he was 31.[31]
  • The disease spread to other royal families in Germany, Russia and Spain through Queen Victoria’s two daughters.
  • The best known case of "the royal disease" was Tsarevich Alexei, son of the Russian Czar Nicholas II.

References

  1. ↑ Brinkhous, K. M. (1975). Handbook of hemophilia. Amsterdam New York: Excerpta Medica Sole distributors for the U.S.A. and Canada, American Elsevier Pub. Co. ISBN 9789021920962.
  2. ↑ 2.0 2.1 Rosendaal FR, Smit C, Briët E (February 1991). "Hemophilia treatment in historical perspective: a review of medical and social developments". Ann. Hematol. 62 (1): 5–15. PMID 1903310.
  3. ↑ 3.0 3.1 3.2 3.3 Ingram, G. I. C. (1997). "The history of haemophilia*,†". Haemophilia. 3 (S1): 5–15. doi:10.1111/j.1365-2516.1997.tb00168.x. ISSN 1351-8216.
  4. ↑ Otto JC (July 1996). "An account of an hemorrhagic disposition existing in certain families". Clin. Orthop. Relat. Res. (328): 4–6. PMID 8653976.
  5. ↑ Brinkhous, K. M. (1975). Handbook of hemophilia. Amsterdam New York: Excerpta Medica Sole distributors for the U.S.A. and Canada, American Elsevier Pub. Co. ISBN 9789021920962.
  6. ↑ Krieger, Marie (1920). Über die Atrophie der Menschlichen Organe bei Inanition. Berlin, Heidelberg: Springer Berlin Heidelberg Imprint Springer. ISBN 3662229374.
  7. ↑ Francis, Sir, Bulloch, William (1909). Treasury of human inheritance. London: Cambridge University Press.
  8. ↑ 8.0 8.1 Wright AE (July 1893). "On a Method of Determining the Condition of Blood Coagulability for Clinical and Experimental Purposes, and on the Effect of the Administration of Calcium Salts in Haemophilia and Actual or Threatened Haemorrhage: [Preliminary Communication]". Br Med J. 2 (1700): 223–5. PMC 2422001. PMID 20754381.
  9. ↑ 9.0 9.1 Addis, T. (1911). "The pathogenesis of hereditary hæmophilia". The Journal of Pathology and Bacteriology. 15 (4): 427–452. doi:10.1002/path.1700150402. ISSN 0368-3494.
  10. ↑ 10.0 10.1 10.2 Creveld, S.; Jordan, F. L. J.; Punt, K. (2009). "Deficiency ot Anti-Hemophilic Factor in a Woman, Combined with a Disturbance in Vascular Function.1". Acta Medica Scandinavica. 151 (5): 381–389. doi:10.1111/j.0954-6820.1955.tb10306.x. ISSN 0001-6101.
  11. ↑ "Commentary on and reprint of Patek AJ Jr, Taylor FHL, Hemophilia. II. Some properties of substances obtained from human plasma effective in accelerating coagulation of hemophiliac blood, in Journal of Clinical Investigation (1937) 16:113–124". 2000: 573–585. doi:10.1016/B978-012448510-5.50144-8.
  12. ↑ Hynes HE, Owen CA, Bowie EJ, Thompson JH (March 1969). "Development of the present concept of hemophilia". Mayo Clin. Proc. 44 (3): 193–206. PMID 4887314.
  13. ↑ Pisciotta AV (August 1980). "Concepts of haemostasis and thrombosis: A study of the coagulation defect in hemophilia and in jaundice (Quick, Stanley-Brown and Bancroft 1935). Armand J. Quick (1894-1978)--a short biography". Thromb. Haemost. 44 (1): 1–5. PMID 6999657.
  14. ↑ 14.0 14.1 Brinkhous, K. M. (1947). "Clotting Defect in Hemophilia: Deficiency in a Plasma Factor Required for Platelet Utilization". Experimental Biology and Medicine. 66 (1): 117–120. doi:10.3181/00379727-66-16003. ISSN 1535-3702.
  15. ↑ QUICK AJ (September 1947). "Studies on the enigma of the hemostatic dysfunction of hemophilia". Am. J. Med. Sci. 214 (3): 272–80. PMID 20263163.
  16. ↑ 16.0 16.1 Lane, Samuel (1840). "HÆMORRHAGIC DIATHESIS". The Lancet. 35 (896): 185–188. doi:10.1016/S0140-6736(00)40031-0. ISSN 0140-6736.
  17. ↑ Bergmann, E. v. (2013). Die Schicksale der Transfusion im Letzten Decennium : Rede, Gehalten zur Feier des Stiftungstages der Militärärztlichen Bildungsanstalten am 2. August 1883. Berlin: Springer Berlin Heidelberg. ISBN 9783642619298.
  18. ↑ Ringer, Sydney (1882). "Regarding the Action of Hydrate of Soda, Hydrate of Ammonia, and Hydrate of Potash on the Ventricle of the Frog's Heart". The Journal of Physiology. 3 (3–4): 195–202. doi:10.1113/jphysiol.1882.sp000095. ISSN 0022-3751.
  19. ↑ Tan, SY; Graham, C (2013). "Karl Landsteiner (1868–1943): Originator of ABO blood classification". Singapore Medical Journal. 54 (5): 243–244. doi:10.11622/smedj.2013099. ISSN 0037-5675.
  20. ↑ "Commentary on and reprint of Landsteiner K, Ueber Agglutinationserscheinungen normalen menschlichen Blute [On the agglutination of normal human blood], in Wiener Klinische Wochenschrift (1901) 14:1132–1134". 2000: 769–775. doi:10.1016/B978-012448510-5.50165-5.
  21. ↑ Eckhardt, Christian (1988). Transfusionsmedizin : Grundlagen · Therapie · Methodik. Berlin, Heidelberg: Springer Berlin Heidelberg Imprint Springer. ISBN 9783662106020.
  22. ↑ Ottenberg, Reuben; Kaliski, David (2009). "Die Gefahren der Transfusionen und deren Verhütung". DMW - Deutsche Medizinische Wochenschrift. 39 (46): 2243–2247. doi:10.1055/s-0028-1128886. ISSN 0012-0472.
  23. ↑ 23.0 23.1 Addis, T. (1916). "The effect of intravenous injections of fresh human serum and of phosphated blood, on the coagulation time of the blood in hereditary hemophila". Experimental Biology and Medicine. 14 (1): 19–23. doi:10.3181/00379727-14-14. ISSN 1535-3702.
  24. ↑ 24.0 24.1 Bendien, W. M. (1937). "INVESTIGATIONS ON HEMOPHILIA". Archives of Pediatrics & Adolescent Medicine. 54 (4): 713. doi:10.1001/archpedi.1937.01980040017002. ISSN 1072-4710.
  25. ↑ Macfarlane, R.G.; Barnett, Burgess (1934). "THE HÆMOSTATIC POSSIBILITIES OF SNAKE-VENOM". The Lancet. 224 (5801): 985–987. doi:10.1016/S0140-6736(00)43846-8. ISSN 0140-6736.
  26. ↑ "Commentary on and reprint of Patek AJ Jr, Taylor FHL, Hemophilia. II. Some properties of substances obtained from human plasma effective in accelerating coagulation of hemophiliac blood, in Journal of Clinical Investigation (1937) 16:113–124". 2000: 573–585. doi:10.1016/B978-012448510-5.50144-8.
  27. ↑ Minot GR, Davidson CS, Lewis JH, Tagnon HJ, Taylor FH (September 1945). "THE COAGULATION DEFECT IN HEMOPHILIA: THE EFFECT, IN HEMOPHILIA, OF THE PARENTERAL ADMINISTRATION OF A FRACTION OF THE PLASMA GLOBULINS RICH IN FIBRINOGEN". J. Clin. Invest. 24 (5): 704–7. doi:10.1172/JCI101654. PMC 435506. PMID 16695264.
  28. ↑ Taylor FH, Davidson CS, Tagnon HJ, Adams MA, Macdonald AH, Minot GR (September 1945). "STUDIES IN BLOOD COAGULATION: THE COAGULATION PROPERTIES OF CERTAIN GLOBULIN FRACTIONS OF NORMAL HUMAN PLASMA IN VITRO". J. Clin. Invest. 24 (5): 698–703. doi:10.1172/JCI101653. PMC 435505. PMID 16695263.
  29. ↑ Nilsson, I. M.; Berntorp, E.; Löfqvist, T.; Pettersson, H. (1992). "Twenty-five years' experience of prophylactic treatment in severe haemophilia A and B". Journal of Internal Medicine. 232 (1): 25–32. doi:10.1111/j.1365-2796.1992.tb00546.x. ISSN 0954-6820.
  30. ↑ Pool, Judith Graham; Shannon, Angela E. (1965). "Production of High-Potency Concentrates of Antihemophilic Globulin in a Closed-Bag System". New England Journal of Medicine. 273 (27): 1443–1447. doi:10.1056/NEJM196512302732701. ISSN 0028-4793.
  31. ↑ 31.0 31.1 Ingram, G I (1976). "The history of haemophilia". Journal of Clinical Pathology. 29 (6): 469–479. doi:10.1136/jcp.29.6.469. ISSN 0021-9746.

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