Table 1

Late-onset cTTP presenting in pregnancy

CasePregnancyTreatment givenOutcomes
1 1. 21/40* PI/PEX IUFD 
2. 37/40 PEX 2 weekly from 12 wk and 6 wk postpartum Live birth 
2 1. 20/40  Stillbirth 
2. FTND LDA Live birth 
3. 10/40  Miscarriage 
4. FTND LDA Live 
5. FTND* LDA and LMWH Live 
3 1. 35/40* PEX Live birth but severe developmental delay 
2. 36/40 BPL 8Y from 12/40, 20 U/kg 2 weekly, increasing to 30 U/kg weekly from 25/40; PEX pre- and postdelivery; LDA and LMWH Live birth 
3. 11/40  Miscarriage 
4. 36/40 PEX every 2 wk, increased to weekly from mid–second trimester Live birth 
4 First trimester* 10/7 PEX until TTP remission, continued 3×/wk PEX until delivery; LDA; LMWH Live 34/40 
5 17/40* Daily PEX, LDA, LMWH, steroids, rituximab Live 30+/40 
6† 1. 37/40 Steroids Stillbirth 
2. 32/40 FFP and steroids IUFD 
3. 32/40 Live birth 
4. 33/40 (twins) IUFD 33/40 
7 1. 20/40  IUFD 
2. 31/40* Daily PEX until remission, then weekly until 37/40 and postpartum Live birth 37/40 
3. 23/40 Daily PEX until remission, then every 2 wk until 31/40, PEX increased to twice weekly; 5 PEX postpartum Live birth 38/40 
8 1. 25/40*  IUFD 
2. 35/40 PEX every 2 wk from 12 wk Live birth 
3. 8/40  Miscarriage 
4. 34/40 PEX every 2 wk from 12 wk; changed to BPL 8Y from 20/40 to delivery Live birth 
9 40/40* PEX, LDA Live birth 
10 37/40* PEX, LDA Live birth 
11 1. 14/40  IUFD 
2. 19/40  IUFD 
3. 20/40  IUFD 
4. 6/40  Miscarriage 
5. 36/40* Daily PEX and steroids Live birth 37/40 
6. 37/40 LDA and LMWH throughout; PI from 10/40 every 2 wk until 20 wk, then continued weekly; 1 PEX pre- and postdelivery Live birth 
12 1. 38/40* PEX, LDA Live birth 
2. 37/40 PEX every 2 wk from 12 wk, continued with PI every 2 wk from second trimester Live birth 
13 1. 39/40* PEX Live birth 
2. 38/40 Weekly PI in third trimester, LMWH, and LDA Live birth 
14 1. 27/40 Platelet transfusion Live birth 
2. 34/40* PEX, steroids, LDA, LMWH, IVIg Live birth 
3. 36/40 BPL 8Y ×2/wk throughout pregnancy and 4 wk postpartum; 2× PEX predelivery Live birth 
15 32/40* Daily PEX to TTP remission then weekly until delivery Live birth 37/40 
16 1. 20/40 (twins) IVIg, prednisolone, platelets, PEX IUFD 25/40 
2. 39/40 PI throughout pregnancies; platelets at delivery Live birth 
3. 37/40 Live birth 
4. 37/40* Live birth 
17 14/40* Daily PEX until remission, then weekly PEX to delivery Live birth 35/40 
18 (Known from positive FH) LDA, PI every 2 wk, then weekly from 25/40 37/40 
19 1. 20/40  IUFD 
2. 24/40 (twins)*  IUFD 
3. 6/40  Miscarriage 
4. 37/40 Plasma treatment ×2/wk from 12 wk Live birth 
20 33/40 Steroids Live birth 
21 1. 34/40 (twins) Platelets Live birth 
2. 28/40* PEX Live 29/40 
22 1. 24/40  IUFD 
2. 26/40  IUFD 
3. 27/40  IUFD 
4, 5, 6. 6-12/40  3 miscarriages 
7. 27/40  Live birth 
8. 20/40* LDA, LMWH, PI, PEX IUFD 28/40 
23† 36/40  Live birth 
CasePregnancyTreatment givenOutcomes
1 1. 21/40* PI/PEX IUFD 
2. 37/40 PEX 2 weekly from 12 wk and 6 wk postpartum Live birth 
2 1. 20/40  Stillbirth 
2. FTND LDA Live birth 
3. 10/40  Miscarriage 
4. FTND LDA Live 
5. FTND* LDA and LMWH Live 
3 1. 35/40* PEX Live birth but severe developmental delay 
2. 36/40 BPL 8Y from 12/40, 20 U/kg 2 weekly, increasing to 30 U/kg weekly from 25/40; PEX pre- and postdelivery; LDA and LMWH Live birth 
3. 11/40  Miscarriage 
4. 36/40 PEX every 2 wk, increased to weekly from mid–second trimester Live birth 
4 First trimester* 10/7 PEX until TTP remission, continued 3×/wk PEX until delivery; LDA; LMWH Live 34/40 
5 17/40* Daily PEX, LDA, LMWH, steroids, rituximab Live 30+/40 
6† 1. 37/40 Steroids Stillbirth 
2. 32/40 FFP and steroids IUFD 
3. 32/40 Live birth 
4. 33/40 (twins) IUFD 33/40 
7 1. 20/40  IUFD 
2. 31/40* Daily PEX until remission, then weekly until 37/40 and postpartum Live birth 37/40 
3. 23/40 Daily PEX until remission, then every 2 wk until 31/40, PEX increased to twice weekly; 5 PEX postpartum Live birth 38/40 
8 1. 25/40*  IUFD 
2. 35/40 PEX every 2 wk from 12 wk Live birth 
3. 8/40  Miscarriage 
4. 34/40 PEX every 2 wk from 12 wk; changed to BPL 8Y from 20/40 to delivery Live birth 
9 40/40* PEX, LDA Live birth 
10 37/40* PEX, LDA Live birth 
11 1. 14/40  IUFD 
2. 19/40  IUFD 
3. 20/40  IUFD 
4. 6/40  Miscarriage 
5. 36/40* Daily PEX and steroids Live birth 37/40 
6. 37/40 LDA and LMWH throughout; PI from 10/40 every 2 wk until 20 wk, then continued weekly; 1 PEX pre- and postdelivery Live birth 
12 1. 38/40* PEX, LDA Live birth 
2. 37/40 PEX every 2 wk from 12 wk, continued with PI every 2 wk from second trimester Live birth 
13 1. 39/40* PEX Live birth 
2. 38/40 Weekly PI in third trimester, LMWH, and LDA Live birth 
14 1. 27/40 Platelet transfusion Live birth 
2. 34/40* PEX, steroids, LDA, LMWH, IVIg Live birth 
3. 36/40 BPL 8Y ×2/wk throughout pregnancy and 4 wk postpartum; 2× PEX predelivery Live birth 
15 32/40* Daily PEX to TTP remission then weekly until delivery Live birth 37/40 
16 1. 20/40 (twins) IVIg, prednisolone, platelets, PEX IUFD 25/40 
2. 39/40 PI throughout pregnancies; platelets at delivery Live birth 
3. 37/40 Live birth 
4. 37/40* Live birth 
17 14/40* Daily PEX until remission, then weekly PEX to delivery Live birth 35/40 
18 (Known from positive FH) LDA, PI every 2 wk, then weekly from 25/40 37/40 
19 1. 20/40  IUFD 
2. 24/40 (twins)*  IUFD 
3. 6/40  Miscarriage 
4. 37/40 Plasma treatment ×2/wk from 12 wk Live birth 
20 33/40 Steroids Live birth 
21 1. 34/40 (twins) Platelets Live birth 
2. 28/40* PEX Live 29/40 
22 1. 24/40  IUFD 
2. 26/40  IUFD 
3. 27/40  IUFD 
4, 5, 6. 6-12/40  3 miscarriages 
7. 27/40  Live birth 
8. 20/40* LDA, LMWH, PI, PEX IUFD 28/40 
23† 36/40  Live birth 

FFP, fresh frozen plasma; FH, family history; FTND, full term normal delivery; IUFD, in utero fetal death; IVIg, intravenous immunoglobulin; LDA, low dose aspirin; LMWH, low molecular weight heparin.

*

Denotes the pregnancy when TTP was diagnosed.

†

Denotes retrospective diagnosis.

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