How Is Thrombotic Thrombocytopenic Purpura
Treated?
Thrombotic thrombocytopenic purpura (TTP) can be
fatal or cause lasting damage, such as brain damage or stroke, if its not
treated promptly.
In most cases, TTP occurs suddenly and lasts for
days and weeks, but it can go on for months. Most people recover fully when
treated right away. Relapses (flareups) can occur in 30 to 60 percent of people
with acquired TTP. Flareups also occur in most people with inherited TTP.
Plasma treatments are the most common way to treat
TTP. Other treatments include medicines and surgery. Treatments are done in a
hospital.
Plasma Therapy
Plasma is the liquid part of your blood. It carries
blood cells, hormones, enzymes, and nutrients to your body.
TTP is treated with plasma therapy. This includes
fresh frozen plasma for newborns and children with inherited TTP, and plasma
exchange for people with acquired TTP. Plasma therapy is started in the
hospital as soon as TTP is diagnosed or suspected.
For inherited TTP, fresh frozen plasma is given
through an intravenous (IV) line in a vein (blood vessel). This is done to
replace the missing or changed ADAMTS13 enzyme.
For acquired TTP, plasma exchange (also called
plasmapheresis) is done. This is a lifesaving procedure. It removes antibodies
from the blood that damage your ADAMTS13 enzyme. It also replaces the ADAMTS13
enzyme. If plasma exchange isnt available, you may be given fresh frozen
plasma until it is available.
During plasma exchange, an IV needle or tube is
placed in your arm to remove blood. The blood then goes through a cell
separator, which removes plasma from the blood. The nonplasma part of the blood
is saved, and donated plasma is added to it.
The blood is then put back into you through an IV
line in one of your blood vessels. The time the procedure takes varies, but it
often takes about 2 hours.
Treatments of fresh frozen plasma or plasma exchange
usually continue until your blood tests and signs and symptoms improve. This
can be days or weeks depending on your condition. You will stay in the hospital
during this time.
Some people who recover from TTP have flareups. This
can happen in the hospital or after you go home. If you have a flareup, plasma
infusion or plasma exchange will be restarted.
Other Treatments
Other treatments are used when plasma infusions or
plasma exchange doesnt work well or when flareups occur often.
Medicines are used to slow or stop antibodies from
forming. In acquired TTP, antibodies block the activity of the ADAMTS13 enzyme.
Medicines used to treat TTP include glucocorticoids, vincristine, rituximab,
and cyclosporine A.
Surgery to remove the spleen (an organ in the
abdomen) is sometimes needed. This is because cells in the spleen make
antibodies that block the activity of the ADAMTS13 enzyme.
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