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BMC Pregnancy Childbirth. 2015 Jun 17;15:137. doi: 10.1186/s12884-015-0557-5.

Incidence of obstetrical thrombotic thrombocytopenic purpura in a retrospective study within thrombocytopenic pregnant women. A difficult diagnosis and a treatable disease.

Delmas Y1,2, Helou S3,4, Chabanier P5,6, Ryman A7,8, Pelluard F9,10, Carles D11,12, Boisseau P13, Veyradier A14,15, Horovitz J16,17, Coppo P18,19,20, Combe C21,22,23.

Author information

1
Service de Néphrologie Transplantation Dialyse, Centre Hospitalier Universitaire de Bordeaux, Bordeaux, France. yahsou.delmas@chu-bordeaux.fr.
2
Centre de Compétence des Microangiopathies Thrombotiques, Centre Hospitalier Universitaire de Bordeaux, Bordeaux, France. yahsou.delmas@chu-bordeaux.fr.
3
Service de Néphrologie Transplantation Dialyse, Centre Hospitalier Universitaire de Bordeaux, Bordeaux, France. helou@clinique-delay.fr.
4
Université Bordeaux Segalen, Bordeaux, France. helou@clinique-delay.fr.
5
Centre de Compétence des Microangiopathies Thrombotiques, Centre Hospitalier Universitaire de Bordeaux, Bordeaux, France. pierre.chabanier@chu-bordeaux.fr.
6
Pôle Gynécologie-Obstétrique-et Médecine Foetale, Centre Hospitalier Universitaire de Bordeaux, Bordeaux, France. pierre.chabanier@chu-bordeaux.fr.
7
Centre de Compétence des Microangiopathies Thrombotiques, Centre Hospitalier Universitaire de Bordeaux, Bordeaux, France. anne.ryman@chu-bordeaux.fr.
8
Service d'Hémostase Spécialisée, Centre Hospitalier Universitaire de Bordeaux, Bordeaux, France. anne.ryman@chu-bordeaux.fr.
9
Service d'Anatomie Pathologique, Centre Hospitalier Universitaire de Bordeaux, Bordeaux, France. fanny.pelluard@chu-bordeaux.fr.
10
Université Bordeaux Segalen, Bordeaux, France. fanny.pelluard@chu-bordeaux.fr.
11
Service d'Anatomie Pathologique, Centre Hospitalier Universitaire de Bordeaux, Bordeaux, France. dominique.carles@u-bordeaux.fr.
12
Université Bordeaux Segalen, Bordeaux, France. dominique.carles@u-bordeaux.fr.
13
Service de Génétique Médicale, Centre Hospitalier Universitaire de Nantes Hôtel Dieu, Nantes, France. pierre.boisseau@chu-nantes.fr.
14
Service d'hématologie, Centre Hospitalier Universitaire de Lariboisière, Assistance Publique Hôpitaux de Paris, Université Paris 7 Denis Diderot, Paris, France. agnes.veyradier@lrb.aphp.fr.
15
Centre de Référence des Microangiopathies Thrombotiques, Paris, France. agnes.veyradier@lrb.aphp.fr.
16
Pôle Gynécologie-Obstétrique-et Médecine Foetale, Centre Hospitalier Universitaire de Bordeaux, Bordeaux, France. jacques.horovitz@chu-bordeaux.fr.
17
Université Bordeaux Segalen, Bordeaux, France. jacques.horovitz@chu-bordeaux.fr.
18
Centre de Référence des Microangiopathies Thrombotiques, Paris, France. paul.coppo@sat.aphp.fr.
19
Service d'Hématologie Hôpital Saint Antoine, Assistance Publique Hôpitaux de Paris, Paris, France. paul.coppo@sat.aphp.fr.
20
Université Pierre et Marie Curie (UPMC), Univ Paris 6, Paris, France. paul.coppo@sat.aphp.fr.
21
Service de Néphrologie Transplantation Dialyse, Centre Hospitalier Universitaire de Bordeaux, Bordeaux, France. christian.combe@chu-bordeaux.fr.
22
Centre de Compétence des Microangiopathies Thrombotiques, Centre Hospitalier Universitaire de Bordeaux, Bordeaux, France. christian.combe@chu-bordeaux.fr.
23
Université Bordeaux Segalen, Bordeaux, France. christian.combe@chu-bordeaux.fr.

Abstract

BACKGROUND:

Thrombotic thrombocytopenic Purpura (TTP) defined as ADAMTS-13 (A Disintegrin And Metalloprotease with ThromboSpondin type 1 domain 13) activity <10 % is a rare aetiology of thrombocytopenia during pregnancy, although the precise incidence is unknown. During pregnancy, the diagnosis of TTP is crucial as it has high feto-maternal morbidity-mortality and requires urgent plasma exchange. The purpose of this study was to assess the incidence of TTP retrospectively and to describe case presentations and follow-up.

METHODS:

A monocentric retrospective study (2008-2009) was conducted among pregnant women followed in a tertiary care obstetrical unit who experienced at least one episode of severe thrombocytopenia (platelets ≤75 G/L) during 2008 and 2009. In cases of uncertain aetiology of thrombocytopenia, ADAMTS-13 activity was assessed by the full length technique.

RESULTS:

Among 8,908 deliveries over the 2 year period, 79 women had a platelet count nadir ≤75 G/L. Eighteen had a known aetiology of thrombocytopenia and 11 were lost to follow-up. Among 50 remaining patients, ADAMTS-13 activity was undetectable (<5 %) in 4, consistent with the diagnosis of TTP. Platelet count spontaneously normalized in 3 patients after delivery. None presented focal cerebral involvement. Three of the four, who were primipara patients, had a sustained severe deficiency in the absence of anti-ADAMTS-13 antibodies, and ADAMTS-13 gene sequencing indicated a constitutive deficiency. The fourth, a multipara patient, had an acquired, auto-immune TTP. Placental pathology in the three primipara patients showed severe and non-specific ischemic lesions. Two patients lost their babies shortly after birth. In subsequent pregnancies in these two patients, prophylactic plasma infusion initiated early with increasing volume throughout pregnancy prevented TTP relapse, improved placental pathology, and led to normal delivery.

CONCLUSIONS:

The prevalence of TTP among thrombocytopenic pregnant women is high, up to 5 % in a tertiary unit. Platelet count normalization after delivery does not eliminate TTP. Clinicians should be aware of TTP during pregnancy, and, even if assessed retrospectively, ADAMTS-13 assessment is of particular importance for identifying patients with congenital TTP. In these patients, preventive plasma infusion and/or exchange can dramatically improve foetal prognosis, resulting in successful childbirth.

PMID:
26081109
PMCID:
PMC4469004
DOI:
10.1186/s12884-015-0557-5
[Indexed for MEDLINE]
Free PMC Article

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