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2020 ISTH TTP Pocket Guideline with GPS

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18 Treatment Statement 28 ➤ Women with a history of iTTP require close monitoring by a hematologist with experience in maternal fetal medicine throughout pregnancy. Their risk of relapse is generally considered to be high if they enter pregnancy with a plasma ADAMTS13 activity below the normal range. If ADAMTS13 activity falls significantly (usually <30 IU/dL or 30% of normal), in the absence of clinical relapse, elective TPE and corticosteroids or azathioprine are often considered. ➤ It is good practice to monitor ADAMTS13 activity throughout pregnancy and in the postpartum period. Induction of labor at 36 to 37 weeks gestation is commonly suggested. In the absence of other obstetrical indications for caesarean section, vaginal delivery is considered the preferred method of delivery in pregnant women with a history of TTP. This statement also applies to women with a history of cTTP. Statement 29 ➤ Women with a history of TTP who are planning pregnancy usually receive preconception counselling. Pregnant women with a history of TTP have a high risk of serious complications during pregnancy. They receive plasma infusion, at a dose of 10 to 15 mL/kg weekly or every two weeks, or TPE, depending on the symptoms. Beginning in the second or early third trimester, the frequency of infusion is usually increased to weekly or twice weekly or TPE. Women with a history of TTP require close monitoring by a hematologist with experience in maternal fetal medicine throughout pregnancy. Induction of labor at 36 to 37 weeks gestation is commonly suggested. In the absence of other obstetrical indications for caesarean section, vaginal delivery is considered the preferred method of delivery in pregnant women with a history of TTP. These statements apply to women with a history of either cTTP or iTTP. Statement 30 ➤ We did not systematically search and review the evidence on the effect of aspirin in pregnant women with TTP. Largely based on indirect evidence from other populations, pregnant women with a history of TTP are usually not offered low dose aspirin throughout pregnancy.

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