Table 3

Published reports of pregnancy outcomes following recovery from TTP associated with acquired, severe ADAMTS13 deficiency

ReferenceCountry/YearWomenPregnancies following TTP
17  France/2003 Patient A.
4 episodes of TTP; splenectomy.
Previous pregnancies not described. 
Pregnancy 1: fifth TTP at 37 wk; infant delivered and survived. 
Pregnancy 2: sixth TTP at 36 wk; infant delivered and survived. ADAMTS13 activity <5% with demonstrable ultralarge VWF multimers at delivery. 
18  UK/2006 Patient 3.
4 episodes of TTP with pulmonary emboli.
Previous pregnancies not described. 
Pregnancy 1: Managed with PEX (every 1-2 wk) and dalteparin beginning at 6 wk because of ADAMTS13 activity <5%. Term delivery, infant survived. 
19  Germany/2009 First pregnancy: preembryonic loss.
Second pregnancy: HELLP and first TTP, 20 wk;
severe preeclampsia, 30 wk; delivery, 33 wk;
infant survived. 
Pregnancy 3: voluntary termination of pregnancy at 9 wk when platelet count decreased from 250 000/µL to 185 000/µL and schistocytes were present in the blood smear. ADAMTS13 activity not reported. 
20  UK/2010 Previous ESRD, kidney transplant.
First pregnancy: preeclampsia and first TTP, 22 wk; stillbirth. 
Pregnancy 2: second TTP at 28 wk. ADAMTS13 activity was 75% at 16 wk and remained >15% through 22 wk. At 28 wk, epistaxis, thrombocytopenia, ADAMTS13 6%. PEX and urgent delivery; infant survived. 
21  US/2011 Patient 1.
First TTP during pregnancy, 22 wk; stillbirth. Previous pregnancies not described. 
Pregnancy 2: second TTP at 22 wk; neonatal death. Maintenance cyclosporine begun following pregnancy. 
Pregnancy 3, presented at 17 wk, continued cyclosporine throughout pregnancy; ADAMTS13 15%-26%. Preeclampsia at 24 wk with no signs of TTP; urgent delivery; infant survived. 
Patient 2.
First TTP. Previous pregnancies not described. 
Pregnancy 1, maintenance cyclosporine discontinued. ADAMTS13 <2.5% throughout pregnancy; no prophylactic treatment. Second TTP at 37 wk; infant outcome not described. 
Patient 3.
3 uncomplicated pregnancies, then first TTP. 
Pregnancy 4: ADAMTS13 92%-100%; no prophylactic treatment; no complications; infant survived. 
22  US/2012 5 pregnancies (1 delivery; 4 early pregnancy loss).
1 episode of TTP, association with pregnancy not described. 
Pregnancy 6: prophylactic treatment with PEX and methylprednisolone (50 mg) 3 times per wk. Severe preeclampsia and TTP at 32 wk, urgent delivery; infant survived. 
ReferenceCountry/YearWomenPregnancies following TTP
17  France/2003 Patient A.
4 episodes of TTP; splenectomy.
Previous pregnancies not described. 
Pregnancy 1: fifth TTP at 37 wk; infant delivered and survived. 
Pregnancy 2: sixth TTP at 36 wk; infant delivered and survived. ADAMTS13 activity <5% with demonstrable ultralarge VWF multimers at delivery. 
18  UK/2006 Patient 3.
4 episodes of TTP with pulmonary emboli.
Previous pregnancies not described. 
Pregnancy 1: Managed with PEX (every 1-2 wk) and dalteparin beginning at 6 wk because of ADAMTS13 activity <5%. Term delivery, infant survived. 
19  Germany/2009 First pregnancy: preembryonic loss.
Second pregnancy: HELLP and first TTP, 20 wk;
severe preeclampsia, 30 wk; delivery, 33 wk;
infant survived. 
Pregnancy 3: voluntary termination of pregnancy at 9 wk when platelet count decreased from 250 000/µL to 185 000/µL and schistocytes were present in the blood smear. ADAMTS13 activity not reported. 
20  UK/2010 Previous ESRD, kidney transplant.
First pregnancy: preeclampsia and first TTP, 22 wk; stillbirth. 
Pregnancy 2: second TTP at 28 wk. ADAMTS13 activity was 75% at 16 wk and remained >15% through 22 wk. At 28 wk, epistaxis, thrombocytopenia, ADAMTS13 6%. PEX and urgent delivery; infant survived. 
21  US/2011 Patient 1.
First TTP during pregnancy, 22 wk; stillbirth. Previous pregnancies not described. 
Pregnancy 2: second TTP at 22 wk; neonatal death. Maintenance cyclosporine begun following pregnancy. 
Pregnancy 3, presented at 17 wk, continued cyclosporine throughout pregnancy; ADAMTS13 15%-26%. Preeclampsia at 24 wk with no signs of TTP; urgent delivery; infant survived. 
Patient 2.
First TTP. Previous pregnancies not described. 
Pregnancy 1, maintenance cyclosporine discontinued. ADAMTS13 <2.5% throughout pregnancy; no prophylactic treatment. Second TTP at 37 wk; infant outcome not described. 
Patient 3.
3 uncomplicated pregnancies, then first TTP. 
Pregnancy 4: ADAMTS13 92%-100%; no prophylactic treatment; no complications; infant survived. 
22  US/2012 5 pregnancies (1 delivery; 4 early pregnancy loss).
1 episode of TTP, association with pregnancy not described. 
Pregnancy 6: prophylactic treatment with PEX and methylprednisolone (50 mg) 3 times per wk. Severe preeclampsia and TTP at 32 wk, urgent delivery; infant survived. 

Articles are described by the year of publication and the country of origin. Women’s risks for pregnancy complications following TTP (age at pregnancy, race, BMI, chronic hypertension, previous preeclampsia) were not reported.

ESRD, end-stage renal disease; PEX, plasma exchange; VWF, von Willebrand factor.

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