Acquired von Willebrand disease associated with free lambda light chain monoclonal gammopathy, normal bleeding time and response to prednisone

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Abstract

We report a case of acquired von Willebrand's syndrome with severe gastrointestinal bleeding and associated free monoclonal lambda light chains. The patient had a rapid sustained clinical and laboratory response to the administration of prednisone. Of note in this patient was the occurrence of angiodysplasia which has previously been reported in association with acquired von Wiliebrand's syndrome. No inhibitors of VWF:Ag, VWF:RCoF, or factor VIII:C were detected by mixing studies and the bleeding time was normal. Very few high molecular weight von Wiliebrand multimers were present prior to prednisone; however, the pattern reverted to a normal distribution following treatment. In appropriate atients with acquired von Willebrand's syndrome and monoclonal para-proteins, a trial of prednisone may be indicated.

Original languageEnglish (US)
Pages (from-to)560-562
Number of pages3
JournalPostgraduate Medical Journal
Volume66
Issue number777
DOIs
StatePublished - 1990
Externally publishedYes

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von Willebrand Diseases
Paraproteinemias
Bleeding Time
Prednisone
Light
Angiodysplasia
Normal Distribution
Factor VIII
Molecular Weight
Hemorrhage
Proteins
Therapeutics

ASJC Scopus subject areas

  • Public Health, Environmental and Occupational Health
  • Epidemiology
  • Medicine(all)

Cite this

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title = "Acquired von Willebrand disease associated with free lambda light chain monoclonal gammopathy, normal bleeding time and response to prednisone",
abstract = "We report a case of acquired von Willebrand's syndrome with severe gastrointestinal bleeding and associated free monoclonal lambda light chains. The patient had a rapid sustained clinical and laboratory response to the administration of prednisone. Of note in this patient was the occurrence of angiodysplasia which has previously been reported in association with acquired von Wiliebrand's syndrome. No inhibitors of VWF:Ag, VWF:RCoF, or factor VIII:C were detected by mixing studies and the bleeding time was normal. Very few high molecular weight von Wiliebrand multimers were present prior to prednisone; however, the pattern reverted to a normal distribution following treatment. In appropriate atients with acquired von Willebrand's syndrome and monoclonal para-proteins, a trial of prednisone may be indicated.",
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T1 - Acquired von Willebrand disease associated with free lambda light chain monoclonal gammopathy, normal bleeding time and response to prednisone

AU - Stewart, Alexander Keith

AU - Glynn, M. F X

PY - 1990

Y1 - 1990

N2 - We report a case of acquired von Willebrand's syndrome with severe gastrointestinal bleeding and associated free monoclonal lambda light chains. The patient had a rapid sustained clinical and laboratory response to the administration of prednisone. Of note in this patient was the occurrence of angiodysplasia which has previously been reported in association with acquired von Wiliebrand's syndrome. No inhibitors of VWF:Ag, VWF:RCoF, or factor VIII:C were detected by mixing studies and the bleeding time was normal. Very few high molecular weight von Wiliebrand multimers were present prior to prednisone; however, the pattern reverted to a normal distribution following treatment. In appropriate atients with acquired von Willebrand's syndrome and monoclonal para-proteins, a trial of prednisone may be indicated.

AB - We report a case of acquired von Willebrand's syndrome with severe gastrointestinal bleeding and associated free monoclonal lambda light chains. The patient had a rapid sustained clinical and laboratory response to the administration of prednisone. Of note in this patient was the occurrence of angiodysplasia which has previously been reported in association with acquired von Wiliebrand's syndrome. No inhibitors of VWF:Ag, VWF:RCoF, or factor VIII:C were detected by mixing studies and the bleeding time was normal. Very few high molecular weight von Wiliebrand multimers were present prior to prednisone; however, the pattern reverted to a normal distribution following treatment. In appropriate atients with acquired von Willebrand's syndrome and monoclonal para-proteins, a trial of prednisone may be indicated.

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