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2020 ISTH TTP Pocket Guideline with GPS

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17 Statement 24 ➤ In some institutions, women with a decreased ADAMTS13 activity (e.g., <10 IU/dL) prior to or at the onset of pregnancy are offered elective rituximab therapy, with the goal to eliminate ADAMTS13 autoantibodies and normalize ADAMTS13 activity before conception. Evidence of increasing ADAMTS13 activity may be sufficient to consider a lower risk of relapse in women with TTP. Statement 25 ➤ Patients treated with rituximab are usually asked to wait for 6-12 months following rituximab administration before trying to conceive; normalization of CD19 lymphocyte levels and undetectable serum rituximab levels are often used as evidence of "drug washout." Global drug safety databases suggest that rituximab is associated with few congenital malformations or neonatal infections, and the scant case reports of its use in patients with TTP did not report maternal or neonatal toxicity. However, women should be clearly informed that the evidence about the safety and efficacy of rituximab in pregnancy is extremely limited and inconclusive. Statement 26 ➤ Pregnant women with a history of either cTTP or iTTP are usually closely monitored by a hematologist and an obstetrician with experience in maternal fetal medicine/perinatology. The panel supports the involvement of clinicians with expertise in TTP in the care of pregnant women with a history of TTP. Complete blood counts are usually monitored at least monthly. Plasma ADAMTS13 activity is usually monitored monthly or every two to three months at least. (More frequent monitoring tends to occur if the ADAMTS13 activity begins to drop.) Statement 27 ➤ TTP presenting in pregnancy generally merits transfer to a specialist center with hematologist, obstetrician, and transfusion medicine specialists, and TPE capabilities, for comprehensive, definitive care. As in suspected iTTP in non-pregnant patients, daily TPE is generally initiated as soon as possible with fresh frozen plasma, cryopoor plasma, or solvent detergent treated plasma as the replacement fluid. The volume of replacement fluid is usually 1-1.5X plasma volume (i.e., 40-60 ml/kg) every 24 hours.

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