molecular abnormality of clotting factor VIII.
Unlike HEMOPHILIAA, which also results from clot-
ting factor VIII deficiency, von Willebrand’s dis-
ease affects both men and women equally. Its
inheritance pattern is autosomal dominant, mean-
ing a child can acquire the condition when only
one parent has the defective GENE. Von Wille-
brand’s disease is the most common bleeding dis-
order in the United States, affecting about 1
percent of the American population. The condi-
tion is mild in most people, though severe trauma
or major surgery may cause life-threatening hem-
orrhage especially in people who do not know
they have the disorder.
Symptoms and Diagnostic Path
The most common symptom of von Willebrand’s
disease is somewhat prolonged bleeding with cuts,
wounds, dental procedures, and surgeries. Easy
bruising, frequent nosebleeds (EPISTAXIS), and
bleeding gums are also common symptoms. Some
people may periodically develop PETECHIAE, pin-
point hemorrhages beneath the surface of the SKIN
that have the appearance of a RASH. Women who
have von Willebrand’s disease may have unusually
heavy menstrual bleeding. The diagnostic path
includes blood tests to measure clotting times,
PLATELET AGGREGATION, and the level of von
Willebrand factor multimers in the blood. The
results of these diagnostic blood tests are usually
conclusive for the diagnosis.
Treatment Options and Outlook
No treatment is necessary for people who have
mild symptoms, though anyone diagnosed with
von Willebrand’s disease should carry or wear
identification that alerts emergency medical per-
sonnel to the condition. Treatment before sched-
uled surgeries or for bleeding due to trauma is
administration of PLASMA cryoprecipitate, which
contains concentrated CLOTTING FACTORS, or puri-
fied factor VIII concentrate.People who have von Willebrand’s dis-
ease should not take NONSTEROIDAL ANTI-
INFLAMMATORY DRUGS(NSAIDS), including
aspirin, as these medications further
decrease PLATELET AGGREGATION and
increase bleeding.Risk Factors and Preventive Measures
As von Willebrand’s disease is hereditary, family
history is the only known risk factor. People who
have von Willebrand’s disease may choose GENETIC
COUNSELINGbefore deciding to conceive children.
Most people who have von Willebrand’s disease
experience little interference with QUALITY OF LIFE.
The condition generally remains stable throughout
life. Other health conditions that affect bleeding
may result in a compound effect to produce more
intense symptoms than either condition alone
would otherwise manifest.
See also COAGULATION; GENETIC DISORDERS.174 The Blood and Lymph