Featured Research

from universities, journals, and other organizations

Suppressing Immune System Reverses Otherwise Untreatable Case Of Blood Disease

Date:
January 22, 2003
Source:
Washington University School Of Medicine
Summary:
Treatment with two medications that suppress the immune system, rituximab and cyclophosphamide, appears to have cured one woman of an otherwise untreatable case of the blood disease known as thrombotic thrombocytopenic purpura (TTP). The findings support the theory that TTP is an autoimmune disease, and not only provide insight into diagnosis and treatment, but also reveal clues about blood clotting and autoimmune diseases in general.

St. Louis, Jan. 21, 2003 -- Treatment with two medications that suppress the immune system, rituximab and cyclophosphamide, appears to have cured one woman of an otherwise untreatable case of the blood disease known as thrombotic thrombocytopenic purpura (TTP). The findings support the theory that TTP is an autoimmune disease, and not only provide insight into diagnosis and treatment, but also reveal clues about blood clotting and autoimmune diseases in general.

"In this particular patient who did not respond to standard therapy, immunosuppression seems to have been successful," says Morey A. Blinder, M.D., associate professor of medicine and of pathology and immunology at Washington University School of Medicine in St. Louis. "These results are promising for others suffering from similarly resistant cases of TTP."

Blinder led the study, in conjunction with J. Evan Sadler, M.D, Ph.D., professor of medicine and of biochemistry and molecular biophysics. Their findings appear in the Jan. 21 issue of the journal Annals of Internal Medicine.

TTP is a blood disorder that affects an estimated 3,000 Americans each year, most of whom are women of childbearing age. Prior to the early 1980s, the prognosis was grim: The risk of dying from complications of the disease such as heart attack or stroke was as high as 90 percent. And because the disease is so rare, it continues to be misdiagnosed and untreated.

Today, most patients who are diagnosed accurately with TTP are successfully treated with plasmapheresis, in which an individual's blood is swapped for healthy blood in a daily process similar to dialysis for kidney failure. But plasmapheresis does not target the underlying problem, which is believed to be similar to autoimmune diseases such as lupus, in which the immune system attacks a person's own tissues. Therefore, even with daily plasmapheresis, the disease returns in about 25 percent of patients.

In 2000, Sadler's team, in collaboration with investigators at the University of Washington in Seattle, identified a protein in the bloodstream called von Willebrand factor-cleaving protease and found that it either is missing or abnormal in people with TTP, presumably as a result of disruption by the immune system. Without it, the protein called von Willebrand factor is not regulated and therefore sticks to itself, forming large clumps, or blood clots, that often lead to stroke or heart attack.

"The discovery of this protein really helps us understand the mechanism of blood clotting in general and how important von Willebrand factor is," says Blinder. "Also, we hope to use this knowledge to develop a definitive test for TTP so that it can be more easily diagnosed and more effectively treated. It also may be possible to genetically engineer the protein for infusion, similar to the use of insulin for diabetes."

To prevent the immune system from destroying or disrupting this essential cleaving protease, the Washington University team tested two drugs already shown to suppress the immune system. In October 2001, after 19 months of relapsing disease despite extensive plasmapheresis, the team gave one 42-year-old woman with severe TTP two drugs – rituximab and cyclophosphamide, both known anti-cancer drugs. Her symptoms and blood levels improved and continue to be stable to date.

"This may not be a public health issue like AIDS or breast cancer, but the fact that first this disease was almost always life-threatening and now may be curable is really important," says Blinder. "And now that we're really beginning to understand the disease itself, it will help us diagnose and treat TTP and will provide insight into blood clotting and how immune diseases work in general."

###

Zheng X, Pallera AM, Goodnough LT, Sadler JE, Blinder MA. Remission of chronic thrombotic thrombocytopenic purpura after treatment with cyclophosphamide and rituximab. Annals of Internal Medicine, vol. 138, Jan. 21, 2003.

Funding from the National Institutes of Health and the Howard Hughes Medical Institute supported this research.

The full-time and volunteer faculty of Washington University School of Medicine are the physicians and surgeons of Barnes-Jewish and St. Louis Children's hospitals. The School of Medicine is one of the leading medical research, teaching and patient-care institutions in the nation. Through its affiliations with Barnes-Jewish and St. Louis Children's hospitals, the School of Medicine is linked to BJC HealthCare.


Story Source:

The above story is based on materials provided by Washington University School Of Medicine. Note: Materials may be edited for content and length.


Cite This Page:

Washington University School Of Medicine. "Suppressing Immune System Reverses Otherwise Untreatable Case Of Blood Disease." ScienceDaily. ScienceDaily, 22 January 2003. <www.sciencedaily.com/releases/2003/01/030122072426.htm>.
Washington University School Of Medicine. (2003, January 22). Suppressing Immune System Reverses Otherwise Untreatable Case Of Blood Disease. ScienceDaily. Retrieved October 22, 2014 from www.sciencedaily.com/releases/2003/01/030122072426.htm
Washington University School Of Medicine. "Suppressing Immune System Reverses Otherwise Untreatable Case Of Blood Disease." ScienceDaily. www.sciencedaily.com/releases/2003/01/030122072426.htm (accessed October 22, 2014).

Share This



More Health & Medicine News

Wednesday, October 22, 2014

Featured Research

from universities, journals, and other organizations


Featured Videos

from AP, Reuters, AFP, and other news services

Orthodontist Mom Jennifer Salzer on the Best Time for Braces

Orthodontist Mom Jennifer Salzer on the Best Time for Braces

Working Mother (Oct. 22, 2014) Is your child ready? Video provided by Working Mother
Powered by NewsLook.com
U.S. Issues Ebola Travel Restrictions, Are Visa Bans Next?

U.S. Issues Ebola Travel Restrictions, Are Visa Bans Next?

Newsy (Oct. 22, 2014) Now that the U.S. is restricting travel from West Africa, some are dropping questions about a travel ban and instead asking about visa bans. Video provided by Newsy
Powered by NewsLook.com
US to Track Everyone Coming from Ebola Nations

US to Track Everyone Coming from Ebola Nations

AP (Oct. 22, 2014) Stepping up their vigilance against Ebola, federal authorities said Wednesday that everyone traveling into the US from Ebola-stricken nations will be monitored for symptoms for 21 days. (Oct. 22) Video provided by AP
Powered by NewsLook.com
Doctors Help Paralysed Man Walk Again, Patient in Disbelief

Doctors Help Paralysed Man Walk Again, Patient in Disbelief

AFP (Oct. 22, 2014) Polish doctors describe how they helped a paralysed man walk again, with the patient in disbelief at the return of sensation to his legs. Duration: 1:04 Video provided by AFP
Powered by NewsLook.com

Search ScienceDaily

Number of stories in archives: 140,361

Find with keyword(s):
Enter a keyword or phrase to search ScienceDaily for related topics and research stories.

Save/Print:
Share:

Breaking News:

Strange & Offbeat Stories


Health & Medicine

Mind & Brain

Living & Well

In Other News

... from NewsDaily.com

Science News

Health News

Environment News

Technology News



Save/Print:
Share:

Free Subscriptions


Get the latest science news with ScienceDaily's free email newsletters, updated daily and weekly. Or view hourly updated newsfeeds in your RSS reader:

Get Social & Mobile


Keep up to date with the latest news from ScienceDaily via social networks and mobile apps:

Have Feedback?


Tell us what you think of ScienceDaily -- we welcome both positive and negative comments. Have any problems using the site? Questions?
Mobile: iPhone Android Web
Follow: Facebook Twitter Google+
Subscribe: RSS Feeds Email Newsletters
Latest Headlines Health & Medicine Mind & Brain Space & Time Matter & Energy Computers & Math Plants & Animals Earth & Climate Fossils & Ruins