16
Treatment
Statement 21
➤ We did not systematically search and review the evidence on
intravenous immunoglobulin (IVIG) for TTP patients. IVIG is generally
not used in TTP. Its efficacy is unknown, and adverse reactions to
this product mimic thrombotic, neurologic, and renal manifestations
of TTP.
Statement 22
➤ We did not systematically search and review the evidence on
corticosteroid dose, dose adjustment or dose tapering in TTP. In
patients with TTP, high doses of glucocorticoids (prednisone, 1 mg/
kg per day orally, or methylprednisolone, 125 mg IV two to four times
daily) are usually used as an initial regimen. If the platelet count
does not increase within three to four days of initiation, higher doses
of glucocorticoids are usually used. High doses are usually continued
until the platelet count has recovered and TPE is stopped. When
platelet count recovery is sustained (e.g., after five to seven days),
glucocorticoids are usually tapered and discontinued over three
weeks. Tapering may be delayed or slowed based on platelet count,
ADAMTS13 results, and/or neurological symptoms.
SECTION IV. TTP and Women's Health
➤ The following statements pertain to women's health issues in patients
with TTP: perinatal care; contraception; and pregnancy counselling.
➤ The following statements apply to women with a history of TTP
Statement 23
➤ Women with a history of TTP who are planning pregnancy usually
receive preconception counselling. The panel acknowledged the
importance of offering counselling to all women with TTP who are
considering pregnancy. The risks of TTP, a rare disease with a
somewhat unpredictable course, must be discussed. The patient's
individual values and preferences must also be considered.
Pregnancy can trigger TTP relapse, resulting in an increased risk
of maternal and fetal morbidity. It is difficult to predict who may
experience relapse during pregnancy. A normal ADAMTS13 activity
at the onset of pregnancy in patients with history of TTP may be
associated with a reduced risk of relapse. A decreased ADAMTS13
activity (e.g., <10 IU/dL) at the onset of pregnancy may be associated
with an increased risk of relapse.
