Pregnancy‐induced thrombocytopenia and TTP, and the risk of fetal death, in Upshaw–Schulman syndrome: a series of 15 pregnancies in 9 genotyped patients

@article{Fujimura2009PregnancyinducedTA,
  title={Pregnancy‐induced thrombocytopenia and TTP, and the risk of fetal death, in Upshaw–Schulman syndrome: a series of 15 pregnancies in 9 genotyped patients},
  author={Yoshihiro Fujimura and Masanori Matsumoto and Koichi Kokame and Ayami Isonishi and Kenji Soejima and Nobu Akiyama and Junji Tomiyama and Kazuhiko Natori and Yasunobu Kuranishi and Yutaka Imamura and Nobumasa Inoue and Satoshi Higasa and Masako Seike and Teruhiko Kozuka and Masamichi Hara and Hideo Wada and Mitsuru Murata and Yasuo Ikeda and Toshiyuki Miyata and James N George},
  journal={British Journal of Haematology},
  year={2009},
  volume={144}
}
Upshaw–Schulman syndrome (USS) is a congenital thrombotic thrombocytopenic purpura (TTP) due to mutations in the gene that encodes for ADAMTS13 (ADAMTS13), but its clinical signs may be mild or absent during childhood. We have identified 37 patients with USS (24 females, 13 males) belonging to 32 families. The nine women from six families who were diagnosed during their first pregnancy are the focus of this report. Six of the nine women had episodes of thrombocytopenia during childhood… Expand
Case of maternal and fetal deaths due to severe congenital thrombotic thrombocytopenic purpura (Upshaw–Schulman syndrome) during pregnancy
TLDR
A 23‐year‐old nulliparous Japanese woman died due to severe, rapid progression of TTP with intrauterine fetal death at 20 weeks of gestation after its onset, even though she underwent intensive treatment which included plasma exchange. Expand
Successful management of a planned pregnancy in severe congenital thrombotic thrombocytopaenic purpura: the Upshaw–Schulman syndrome
TLDR
The patient decided to try for a new pregnancy under close monitoring of her condition, and a plan was made for regular check-ups of foetus and mother. Expand
Thrombotic thrombocytopenic purpura and pregnancy: presentation, management, and subsequent pregnancy outcomes.
TLDR
Careful diagnosis, monitoring, and treatment in congenital and acquired TTP have assisted in excellent pregnancy outcomes and must be differentiated by ADAMTS13 analysis. Expand
Unexpected frequency of Upshaw-Schulman syndrome in pregnancy-onset thrombotic thrombocytopenic purpura.
TLDR
Pregnancy-onset TTP defines a specific subgroup of patients with a strong genetic background that is associated with very high TTP relapse and abortion rates, whereas prophylactic plasmatherapy was beneficial for both the mother and the baby. Expand
A successfully treated case of an acute presentation of congenital thrombotic thrombocytopenic purpura (Upshaw–Schulman syndrome) with decreased ADAMTS13 during late stage of pregnancy
TLDR
Although the mother's platelet count and ADAMTS13 activity decreased temporarily, both values increased following fresh frozen plasma transfusion, and this case showed interesting aspects of congenital thrombocytopenic purpura (Upshaw–Schulman syndrome) in pregnancy. Expand
Late onset and pregnancy-induced congenital thrombotic thrombocytopenic purpura.
TLDR
Genetic analysis of the ADAMTS13 gene is important in TTP patients of all ages if an ADAM TS13 inhibitor has been excluded, as well as in patients whose elder sister died in 1977 during her second pregnancy from a second acute TTP episode. Expand
Incidence of obstetrical thrombotic thrombocytopenic purpura in a retrospective study within thrombocytopenic pregnant women. A difficult diagnosis and a treatable disease
TLDR
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. Expand
Upshaw-Schulman syndrome and pregnancy: successful management with plasma infusions
TLDR
A 22-year-old female with a history of TTP, complicated by a prolonged hospitalization at age 17, who was successfully managed during pregnancy with regular plasma infusions and who had an uneventful delivery is presented. Expand
Novel ADAMTS13 mutations in an obstetric patient with Upshaw‐Schulman syndrome
TLDR
A patient diagnosed with USS following retinal detachments and intrauterine fetal demise at 34 weeks of gestation is presented, highlighting the importance of molecular analysis of the ADAMTS13 gene in patients and family members when the severe ADAMts13 deficiency does not appear to be autoimmune in nature. Expand
Pregnancy outcomes following recovery from acquired thrombotic thrombocytopenic purpura.
TLDR
With prospective complete follow-up, recurrent TTP complicating subsequent pregnancies in Oklahoma patients is uncommon, but the occurrence of preeclampsia may be increased. Expand
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TLDR
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TLDR
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TLDR
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Plasma of patients with Upshaw–Schulman syndrome, a congenital deficiency of von Willebrand factor‐cleaving protease activity, enhances the aggregation of normal platelets under high shear stress
TLDR
The present results clearly indicate that thrombocytopenia in USS patients is caused by a combination of the presence of UL‐VWFMs, platelets and high shear stress generated in the microcirculation. Expand
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