Willebrand v.-jürgens disease D68.0
Synonym(s)
Angiohemophilia; Hereditary thrombopathy; Pseudohaemophilia
History
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Minot, 1928; von Willebrand, 1926; Jürgens, 1933
Definition
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Autosomal dominant inherited hemorrhagic diathesis with prolonged bleeding time and absence of von Willebrand factor.
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Manifestation
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Hemorrhages occur especially in spring and autumn.
Clinic
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Bleeding of the skin and mucous membranes, petechiae, ecchymoses, hyper- and polymenorrhoea, rarely joint bleeding. Severe bleeding during trauma or surgery.
Laboratory
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Prolongation of bleeding time, level of the Ristocetin co-factor less than 80%. Factor VIII 25-15%; characteristic temporary fluctuations.
Differential diagnosis
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Idiopathic thrombocytopenic purpura, thrombocytopenia, anticoagulant therapy.
Internal therapy
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- Desmopressin (Minirin) 0.4 μg/kg bw/day i.v. Control of bleeding time and factor VIII: RCoF increase.
- In severe cases, factor VIII substitution: RCoF-containing concentrate (e.g. Haemate HS) 20-40 IU/kg bw.
Progression/forecast
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Cheap. Improvement of bleeding tendency after the age of 20.
Literature
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- Minot F (1852) On Hemorrhage from the Umbilicus in New-born Infants, with an Analysis of Forty-six Cases. Am J Medical Sciences 24: 310-320
- Minot GR (1928) Familial hemorrhagic condition associated with prolongation of bleeding time. Am J Medical Science 175: 301-306
- by Willebrand EA (1926) Hereditary pseudohemofili. Finska Läkaresällskapets Handlingar 68: 87-112
- by Willebrand EA (1931) About hereditary pseudo-hemophilia. Acta Med Scand 76: 521-550
- von Willebrand EA, Jürgens R (1933) About a new hereditary bleeding scourge: constitutional thrombopathy. German archive Klin Med (Leipzig) 175: 453-483
- von Willebrand EA, Jürgens R (1933) On a new hemophilia: the constitutional thrombopathy Klin Wochenschr (Berlin) 12: 414-417
Recommended articles
Incoming links (4)
Angiohemophilia; Hereditary thrombopathy; Pseudohaemophilia; Thromboplastin time partial;Outgoing links (5)
Ecchymoses; Hemorrhagic diathesis; Hemorrhagy; Purpura idiopathic thrombocytopenic; Thrombocytopenia;Disclaimer
Please ask your physician for a reliable diagnosis. This website is only meant as a reference.
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Prof. Dr. med. Peter Altmeyer