Willebrand v.-jürgens diseaseD68.0

Author:Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Angiohemophilia; Hereditary thrombopathy; Pseudohaemophilia

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HistoryThis section has been translated automatically.

Minot, 1928; von Willebrand, 1926; Jürgens, 1933

DefinitionThis section has been translated automatically.

Autosomal dominant inherited hemorrhagic diathesis with prolonged bleeding time and absence of von Willebrand factor.

ManifestationThis section has been translated automatically.

Hemorrhages occur especially in spring and autumn.

Clinical featuresThis section has been translated automatically.

Bleeding of the skin and mucous membranes, petechiae, ecchymoses, hyper- and polymenorrhoea, rarely joint bleeding. Severe bleeding during trauma or surgery.

LaboratoryThis section has been translated automatically.

Prolongation of bleeding time, level of the Ristocetin co-factor less than 80%. Factor VIII 25-15%; characteristic temporary fluctuations.

Differential diagnosisThis section has been translated automatically.

Idiopathic thrombocytopenic purpura, thrombocytopenia, anticoagulant therapy.

Internal therapyThis section has been translated automatically.

  • 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/forecastThis section has been translated automatically.

Cheap. Improvement of bleeding tendency after the age of 20.

LiteratureThis section has been translated automatically.

  1. 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
  2. Minot GR (1928) Familial hemorrhagic condition associated with prolongation of bleeding time. Am J Medical Science 175: 301-306
  3. by Willebrand EA (1926) Hereditary pseudohemofili. Finska Läkaresällskapets Handlingar 68: 87-112
  4. by Willebrand EA (1931) About hereditary pseudo-hemophilia. Acta Med Scand 76: 521-550
  5. von Willebrand EA, Jürgens R (1933) About a new hereditary bleeding scourge: constitutional thrombopathy. German archive Klin Med (Leipzig) 175: 453-483
  6. von Willebrand EA, Jürgens R (1933) On a new hemophilia: the constitutional thrombopathy Klin Wochenschr (Berlin) 12: 414-417

Authors

Last updated on: 29.10.2020

Author: Prof. Dr. med. Peter Altmeyer