Thrombotic thrombocytopenic purpura: medical and biological monitoring of six pregnancies

Ducloy-Bouthors, A.-S.; Caron, Claudine; Subtil, D.; François, Patrice; Tournoys, Antoine; Wibau, Bénédicte; Krivosic-Horber, R

doi:10.1016/s0301-2115(03)00199-4

Cited by 44 publications

(39 citation statements)

References 8 publications

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“…Analysis of the literature revealed 17 detailed case reports of pregnancy-associated acquired TTP in which ADAMTS13 was documented. 10,19,23,24,28,[41][42][43][44][45][46] Presentation and short-term outcome of our patients were similar to those reported previously, showing a great majority of first pregnancies, a higher prevalence during the second half of pregnancy, the presence of a previously diagnosed autoimmune context (ie, lupus, antiphospholipid antibody syndrome, or acquired TTP) in approximately 25% of cases, and the efficacy of curative PT (however, with a frequent need for additional immune modulators). 47 In our patients, the stillbirth rate remains very high (approximately 70%; 95% CI, 52%-81%) and mainly related to the high frequency of TTP before 28 weeks of gestation, whereas episodes occurring during the third trimester of pregnancy were associated with a good baby prognosis.…”

Section: Org Fromsupporting

confidence: 86%

Unexpected frequency of Upshaw-Schulman syndrome in pregnancy-onset thrombotic thrombocytopenic purpura

Moatti-Cohen

Garrec

Wolf

et al. 2012

Blood

217

229

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to identify all women who were pregnant at their initial TTP presentation. Among 592 adulthood-onset TTP patients with a severe ADAMTS13 deficiency, 42 patients with a pregnancy-onset TTP were included. Surprisingly, the proportion of USS patients (n ‫؍‬ 10 of 42 patients [24%]; confidence interval, 13%-39%) with pregnancy-onset TTP was much higher than that in adulthood-onset TTP in general (less than 5%) and was mostly related to a cluster of ADAMTS13 variants. In the present study, subsequent pregnancies in USS patients not given prophylaxis were associated with very high TTP relapse and abortion rates, whereas prophylactic plasmatherapy was beneficial for both the mother and the baby. Pregnancyonset TTP defines a specific subgroup of patients with a strong genetic background. This study was registered at www.clinicaltrials.gov as number

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Section: Org Fromsupporting

confidence: 86%

Unexpected frequency of Upshaw-Schulman syndrome in pregnancy-onset thrombotic thrombocytopenic purpura

Moatti-Cohen

Garrec

Wolf

et al. 2012

Blood

217

229

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“…6 In contrast, a review of 23 previously published cases of pregnancy-related TMA associated with ADAMTS13 deficiency [21][22][23][24][25][26][27][28][29][30] clearly shows this type of TMA occurs predominantly in the late second and third trimesters. This is in accordance with a recent review of 166 pregnancyrelated TTP cases in which the median time of TTP occurrence was 23 weeks.…”

Section: Discussionmentioning

confidence: 97%

Pregnancy-Associated Hemolytic Uremic Syndrome Revisited in the Era of Complement Gene Mutations

Fakhouri¹,

Roumenina²,

François³

et al. 2010

Journal of the American Society of Nephrology

401

395

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In contrast to pregnancy-associated thrombotic thrombocytopenic purpura, the pathogenesis and presentation of pregnancy-associated atypical hemolytic uremic syndrome (P-aHUS) remain ill-defined. We conducted a retrospective study to assess the presentation and outcomes of patients presenting with P-aHUS and the prevalence of alternative C3 convertase dysregulation. P-aHUS occurred in 21 of the 100 adult female patients with atypical HUS, with 79% presenting postpartum. We detected complement abnormalities in 18 of the 21 patients. The outcomes were poor: 62% reached ESRD by 1 month and 76% by last follow-up. The risk for P-aHUS was highest during a second pregnancy. Thirty-five women, 26 (74%) of whom had complement abnormalities, had at least one pregnancy before the onset of a non-pregnancy-related aHUS. Outcomes did not differ between patients with pregnancy-related and non-pregnancy-related aHUS. Mutations in the SCR19-20 domains of factor H were less frequent in P-aHUS patients compared with non-pregnancy-related aHUS. Pregnancies in female patients with complement abnormalities (n ϭ 44) were complicated by fetal loss and preeclampsia in 4.8% and 7.7%, respectively. Better understanding of complement dysregulation in pregnancy complications is essential, especially to guide development of pharmacologic agents to modulate this system.

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“…Our experience is different from the experience described in published reports, in which 6 of 9 subsequent pregnancies continuing beyond 9 weeks (5 of 7 women) were associated with a recurrent episode of TTP. [17][18][19][20][21][22] This may be related to a bias for reporting recurrences of TTP rather than reporting uncomplicated pregnancies.…”

Section: Discussionmentioning

confidence: 99%

“…17,[20][21][22] In 2 of the 6 women, TTP had occurred during their previous pregnancy. 20,21 No postpartum recurrence of TTP was reported, but no postpartum follow-up was described.…”

Section: Oklahoma Registry Datamentioning

confidence: 99%

Pregnancy outcomes following recovery from acquired thrombotic thrombocytopenic purpura

Yang¹,

McIntosh

Reese³

et al. 2014

Blood

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Key Points• Recurrent TTP complicating a subsequent pregnancy may be uncommon; most pregnancies result in normal children.• The frequency of preeclampsia may be increased in pregnancies following recovery from TTP.Pregnancy may precipitate acute episodes of thrombotic thrombocytopenic purpura (TTP), but pregnancy outcomes in women who have recovered from acquired TTP are not well documented. We analyzed pregnancy outcomes following recovery from TTP associated with acquired, severe ADAMTS13 deficiency (ADAMTS13 activity <10%) in women enrolled in the Oklahoma TTP-HUS Registry from 1995 to 2012. We also systematically searched for published reports on outcomes of pregnancies following recovery from TTP associated with acquired, severe ADAMTS13 deficiency. Ten women in the Oklahoma Registry had 16 subsequent pregnancies from 1999 to 2013. Two women had recurrent TTP, which occurred 9 and 29 days postpartum. Five of 16 pregnancies (31%, 95% confidence interval, 11%-59%) in 3 women were complicated by preeclampsia, a frequency greater than US population estimates (2.1%-3.2%). Thirteen (81%) pregnancies resulted in normal children. The literature search identified 382 articles. Only 6 articles reported pregnancies in women who had recovered from TTP associated with acquired, severe ADAMTS13 deficiency, describing 10 pregnancies in 8 women. TTP recurred in 6 pregnancies. Conclusions: With prospective complete follow-up, recurrent TTP complicating subsequent pregnancies in Oklahoma patients is uncommon, but the occurrence of preeclampsia may be increased. Most pregnancies following recovery from TTP in Oklahoma patients result in normal children.

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Thrombotic thrombocytopenic purpura: medical and biological monitoring of six pregnancies

Cited by 44 publications

References 8 publications

Unexpected frequency of Upshaw-Schulman syndrome in pregnancy-onset thrombotic thrombocytopenic purpura

Unexpected frequency of Upshaw-Schulman syndrome in pregnancy-onset thrombotic thrombocytopenic purpura

Pregnancy-Associated Hemolytic Uremic Syndrome Revisited in the Era of Complement Gene Mutations

Pregnancy outcomes following recovery from acquired thrombotic thrombocytopenic purpura

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