Platelet-type von Willebrand's disease; Characterization of a new bleeding disorder

J. L. Miller, A. Castella

Research output: Contribution to journalArticlepeer-review

165 Citations (Scopus)

Abstract

An autosomally transmitted bleeding diathesis sharing some, but not all, features previously described in von Willebrand's disease (vWd) was studied in five patients representing three generations of a single family. Bleeding times in the upper normal range in conjunction with low-normal platelet counts, normal factor VIII coagulant activity and VIII-related antigen, decreased VIII-ristocetin cofactor activity, selective decrease of the higher molecule weight factor VIII/von Willebrand factor (VIII/vWf) multimers, and increased ristocetin-induced platelet agglutination at low ristocetin concentrations were characteristic. Binding of patient VIII/vWF to washed normal platelets was within normal limits, whereas binding of normal VIII/vWF to patient platelets was significantly increased (p < 0.001 at 0.6 mg/ml ristocetin). This disorder accordingly appears to involve an intrinsic platelet abnormality affecting platelet-VIII/vWF interactions. It is proposed that this concept of vWD be broadened to include patients with this abnormality, which may appropriately be called 'Platelet-type von Willebrand's disease'.

Original languageEnglish
Pages (from-to)790-794
Number of pages5
JournalBlood
Volume60
Issue number3
DOIs
Publication statusPublished - 1982
Externally publishedYes

ASJC Scopus subject areas

  • Biochemistry
  • Immunology
  • Hematology
  • Cell Biology

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