Consider von Willebrand Disease (vWD) in teenage patients with menorrhagia during menarche
Consider von Willebrand Disease (vWD) in teenage patients with menorrhagia during menarche: Excerpt from Avoiding Common Pediatric Errors
Lindsey Albrecht, MD
What to Do - Interpret the Data
Menarche is often when the disease first presents in women.
vWDisthemostcommoninheritedbleedingdisorder,withaprevalence
of about 1.3% in the pediatric population. It can be caused by either a qualitative or quantitative defect in von Willebrand factor, a protein that plays an
important role in both platelet adhesion and the transportation of clotting
factor VIII. vWD is inherited in an autosomal manner; there is marked heterogeneity in the extent of bleeding symptoms, given the variance in possible
underlying protein defects. Even in families with identical mutations, the
extent of bleeding may vary. Symptoms of vWD may include easy bruising, heavy postoperative or postpartum bleeding, gingival bleeding, bleeding
following dental procedures or extractions, and menorrhagia (heavy cyclic
menstrual bleeding).
Menorrhagia is reported in the majority of women with vWD and may
in fact be the only manifestation of the disorder. Menorrhagia beginning at
the time of menarche is typical, with one large study showing that 65% of
women with vWD report menorrhagia beginning at menarche (vs. 9% of
women with menorrhagia without vWD). Menorrhagia often affects quality
of life around the time of menstruation, and can be severe enough to result in
red blood cell transfusion or even hysterectomy or other measures to prevent
severe bleeding. In addition tomenorrhagia,women with vWD havea longer
duration of menstrual bleeding per menstrual cycle. Adolescent girls may be
particularly prone to menorrhagia; the concentration of von Willebrand factor increases with age, potentially decreasing menstrual bleeding over time.
Among all women with menorrhagia, studies demonstrate that roughly
15% will have vWD. Smaller percentages may have other inherited forms of
bleedingdisorders,suchasclottingfactordeficienciesorplateletdysfunction.
Given the significant proportion of vWD among women with menorrhagia,
consideration of the disorder is appropriate, especially in the adolescent
female presenting with heavy bleeding at or since the time of menarche.
History taking in a patient suspected of having vWD should include
a detailed menstrual history, including how often the patient changes her
tampon or pad on the heaviest day, how many tampons or pads are used for a
typical menstrual cycle, and the length of a typical menstrual cycle. Because
patients with vWD usually have heavy bleeding in regular cycles, irregular
patterns of bleeding may imply another etiology involving hormonal imbalance. Quality-of-life issues, such as missed days of school, should also be
assessed. A history of bleeding following procedures or interventions needed
for bleeding (e.g., blood cell transfusion) should be elicited. A family history
of bleeding may not be present but should be considered.
Basic coagulation studies are not sufficient as a laboratory investigation,
as most women with vWD will have a normal activated partial prothrombin
time (given adequate concentration and activity of factor VIII to produce a
normal test result). International normalized ratio is typically normal and
is not helpful in making the diagnosis. A von Willebrand profile, including
von Willebrand factor antigen, ristocetin cofactor, and factor VIII level, is
usually sufficient to make the diagnosis. A complete blood count should be
performed to evaluate platelet numbers in patients with heavy bleeding but
will also reveal anemia secondary to blood loss in many menstruating women
with vWD. Additional laboratory testing, of course, should be performed as
appropriate for the clinical situation, because many other scenarios can lead
to heavy menstrual bleeding (i.e., hypothyroidism).
Adolescent patients with menorrhagia beginning at the time of menarche have an increased risk of having vWD. The diagnosis of this disorder
is important because it allows the opportunity for minimization of a patient's bleeding risk prior to surgical procedures or childbirth. Additionally,
because therapeutic options (such as intranasal desmopressin) exist, interventions can lead to the attenuation of monthly symptoms.
Suggested Readings
Kadir RA, Economides DL, Sabin CA, et al. Assessment of menstrual blood loss and gyneco
logical problems in patients with inherited bleeding disorders. Haemophilia. 1999;5:40–48.
Kadir RA, Economides DL, Sabin CA, et al. Frequency of inherited bleeding disorders in
women with menorrhagia. Lancet. 1998;351:485–489.
Kouides PA. Menorrhagia from a haemotologist's point of view. Part I: initial evaluation.
Haemophilia. 2002;8:330–338.
Werner EJ, Broxson EH, Tucker EL, et al. Prevalence of von Willebrand disease in children: a
multiethnic study. J Pediatr. 1993;123:893–898.
Book Source Details
- Book Title: Avoiding Common Pediatric Errors
- Author(s): Anthony D Slonim MD, DrPH; Lisa Marcucci MD
- Year of Publication: 2008
- Copyright Details: Avoiding Common Pediatric Errors, Copyright © 2008 Lippincott Williams & Wilkins.
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Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.
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More About This Book:
Title: Avoiding Common Pediatric Errors
Authors: Anthony D Slonim MD, DrPH; Lisa Marcucci MD
Publisher: Lippincott Williams & Wilkins
Copyright: 2008
ISBN: 0-7817-7489-6
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