Clinical manifestations and complications of childbirth and replacement therapy in 385 Iranian patients with type 3 von Willebrand disease

Research output: Contribution to journalArticle

157 Citations (Scopus)

Abstract

Type 3 is the most severe form of von Willebrand disease (VWD) transmitted as an autosomal recessive trait. We collected data on the clinical manifestations of type 3 VWD by examining 385 patients from 300 Iranian kindreds, who were compared with 100 age-matched patients with severe haemophilia A. Joint and muscle bleeding was less frequent than in haemophiliacs, perhaps because factor VIII levels were in general higher (median value 4% vs. 1% or less). Mucosal tract haemorrhages such as epistaxis and menorrhagia were the most prevalent symptoms in VWD. Post-circumcision and oral cavity bleeding occurred frequently when prophylactic replacement therapy was not carried out or was inadequate. The course of pregnancy was usually uneventful, but increased bleeding occurred at parturition when affected women were treated with replacement therapy for less than 3-4 d. Ten of 385 (2·6%) of these multitransfused patients developed an alloantibody to VWF and 55% are chronically infected with the hepatitis C virus.

Original languageEnglish
Pages (from-to)1236-1239
Number of pages4
JournalBritish Journal of Haematology
Volume111
Issue number4
DOIs
Publication statusPublished - 2000

Fingerprint

Type 3 Von Willebrand's Disease
Parturition
Hemorrhage
Menorrhagia
Isoantibodies
von Willebrand Diseases
Epistaxis
Factor VIII
Hemophilia A
Therapeutics
Hepacivirus
Mouth
Joints
Pregnancy
Muscles

Keywords

  • von Willebrand disease
  • von Willebrand factor

ASJC Scopus subject areas

  • Hematology

Cite this

@article{41b1d37123f34449acd151eeac9f5842,
title = "Clinical manifestations and complications of childbirth and replacement therapy in 385 Iranian patients with type 3 von Willebrand disease",
abstract = "Type 3 is the most severe form of von Willebrand disease (VWD) transmitted as an autosomal recessive trait. We collected data on the clinical manifestations of type 3 VWD by examining 385 patients from 300 Iranian kindreds, who were compared with 100 age-matched patients with severe haemophilia A. Joint and muscle bleeding was less frequent than in haemophiliacs, perhaps because factor VIII levels were in general higher (median value 4{\%} vs. 1{\%} or less). Mucosal tract haemorrhages such as epistaxis and menorrhagia were the most prevalent symptoms in VWD. Post-circumcision and oral cavity bleeding occurred frequently when prophylactic replacement therapy was not carried out or was inadequate. The course of pregnancy was usually uneventful, but increased bleeding occurred at parturition when affected women were treated with replacement therapy for less than 3-4 d. Ten of 385 (2·6{\%}) of these multitransfused patients developed an alloantibody to VWF and 55{\%} are chronically infected with the hepatitis C virus.",
keywords = "von Willebrand disease, von Willebrand factor",
author = "M. Lak and F. Peyvandi and Mannucci, {P. M.}",
year = "2000",
doi = "10.1046/j.1365-2141.2000.02507.x",
language = "English",
volume = "111",
pages = "1236--1239",
journal = "British Journal of Haematology",
issn = "0007-1048",
publisher = "John Wiley & Sons, Ltd (10.1111)",
number = "4",

}

TY - JOUR

T1 - Clinical manifestations and complications of childbirth and replacement therapy in 385 Iranian patients with type 3 von Willebrand disease

AU - Lak, M.

AU - Peyvandi, F.

AU - Mannucci, P. M.

PY - 2000

Y1 - 2000

N2 - Type 3 is the most severe form of von Willebrand disease (VWD) transmitted as an autosomal recessive trait. We collected data on the clinical manifestations of type 3 VWD by examining 385 patients from 300 Iranian kindreds, who were compared with 100 age-matched patients with severe haemophilia A. Joint and muscle bleeding was less frequent than in haemophiliacs, perhaps because factor VIII levels were in general higher (median value 4% vs. 1% or less). Mucosal tract haemorrhages such as epistaxis and menorrhagia were the most prevalent symptoms in VWD. Post-circumcision and oral cavity bleeding occurred frequently when prophylactic replacement therapy was not carried out or was inadequate. The course of pregnancy was usually uneventful, but increased bleeding occurred at parturition when affected women were treated with replacement therapy for less than 3-4 d. Ten of 385 (2·6%) of these multitransfused patients developed an alloantibody to VWF and 55% are chronically infected with the hepatitis C virus.

AB - Type 3 is the most severe form of von Willebrand disease (VWD) transmitted as an autosomal recessive trait. We collected data on the clinical manifestations of type 3 VWD by examining 385 patients from 300 Iranian kindreds, who were compared with 100 age-matched patients with severe haemophilia A. Joint and muscle bleeding was less frequent than in haemophiliacs, perhaps because factor VIII levels were in general higher (median value 4% vs. 1% or less). Mucosal tract haemorrhages such as epistaxis and menorrhagia were the most prevalent symptoms in VWD. Post-circumcision and oral cavity bleeding occurred frequently when prophylactic replacement therapy was not carried out or was inadequate. The course of pregnancy was usually uneventful, but increased bleeding occurred at parturition when affected women were treated with replacement therapy for less than 3-4 d. Ten of 385 (2·6%) of these multitransfused patients developed an alloantibody to VWF and 55% are chronically infected with the hepatitis C virus.

KW - von Willebrand disease

KW - von Willebrand factor

UR - http://www.scopus.com/inward/record.url?scp=0034486136&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0034486136&partnerID=8YFLogxK

U2 - 10.1046/j.1365-2141.2000.02507.x

DO - 10.1046/j.1365-2141.2000.02507.x

M3 - Article

C2 - 11167767

AN - SCOPUS:0034486136

VL - 111

SP - 1236

EP - 1239

JO - British Journal of Haematology

JF - British Journal of Haematology

SN - 0007-1048

IS - 4

ER -