Roanoke Times
Copyright (c) 1995, Landmark Communications, Inc.
DATE: TUESDAY, August 29, 1995 TAG: 9508290010
SECTION: EXTRA PAGE: 1 EDITION: METRO
SOURCE:
DATELINE: LENGTH: Medium
Hemophilia afflicts one man in 10,000, but von Willebrand's may affect as many as one in 100 men and women, often causing frequent nosebleeds, bleeding gums, easy bruising and excessive menstrual flow.
Unlike hemophilia, it is not usually life-threatening.
More than 95 percent of people with von Willebrand's disease never know they have it.
Some have no recognizable symptoms, but others accept their annoying symptoms as normal, particularly if a parent had similar ones. Yet, the failure to diagnose von Willebrand's disease as a cause of very heavy menstrual bleeding, or menorrhagia, can result in unnecessary tests and procedures, including repeated dilation and curettage procedures and hysterectomy, none of which get to the root of the problem.
At the annual meeting of the American College of Obstetricians and Gynecologists this year, an expert committee urged colleagues to think of this condition and test patients for it when the complaint is menorrhagia. But doctors must realize that von Willebrand's disease is also a potentially serious problem for men who, like women, can bleed excessively after a traumatic injury or operation, including tooth extraction.
``Every primary-care physician and those who work in emergency rooms should be alert to this disease,'' said Dr. Jeanne Lusher, a hematologist who is director of the hemophilia program at Wayne State University School of Medicine and Children's Hospital of Michigan in Detroit.
``If any patient is having mucous membrane bleeding, von Willebrand's disease should cross their minds as a possibility.''
Like hemophilia, von Willebrand's disease is hereditary. Hemophilia is caused by a recessive gene passed on to sons by a mother who carries the gene on one of her X chromosomes. (Boys inherit only one X chromosome and only from their mothers.) Although two recessive genes are usually needed for a characteristic to be expressed, in hemophilia the recessive gene is expressed in male children because there is no normal dominant gene to oppose it.
The gene for von Willebrand's disease is a dominant gene, so only one is needed to cause it. In addition, it is carried on an autosome (a nonsex chromosome) and can therefore be transmitted to sons or daughters by either parent.
The gene involved in von Willebrand's disease specifies the production of a protein, von Willebrand factor, that circulates in the blood and is critical to the initial stages of clotting.
Produced by cells that line blood vessel walls, the protein acts like glue, forming a plug of blood platelets to seal off blood flow. It also helps to stabilize factor VIII, the clotting protein often missing in hemophilia.
by CNB