Upshaw-Schulman syndrome diagnosed during pregnancy complicated by reversible cerebral vasoconstriction syndrome

Mariko Tsuda, Motoaki Shiratsuchi, Yasuhiro Nakashima, Motohiko Ikeda, Hiroki Muta, Taisuke Narazaki, Toru Masuda, Daisaku Kimura, Akiko Takamatsu, Masanori Matsumoto, Yoshihiro Fujimura, Koichi Kokame, Takamitsu Matsushima, Yoshihiro Ogawa

Research output: Contribution to journalArticle

Abstract

Upshaw-Schulman syndrome (USS) is an inherited type of thrombotic thrombocytopenic purpura (TTP) that is extremely rare, but often diagnosed during pregnancy. Reversible cerebral vasoconstriction syndrome (RCVS) is the transient stenosis of several cerebral arteries that is frequently diagnosed post-partum. We describe a 28-year-old woman with USS complicated by RCVS after delivery that was treated by plasma exchange with a good outcome. She was referred to our hospital with thunderclap headache, anemia and thrombocytopenia that occurred immediately postpartum. She was diagnosed with TTP and multiple cerebral infarctions. Plasma exchange promptly improved her symptoms on hospital day 3. Moreover, multiple stenoses of cerebral arteries indicating RCVS were resolved. Since her sister also had an episode of thrombocytopenia during pregnancy, inherited TTP was suspected and genetic analyses confirmed USS. Pregnancy is a risk for not only TTP, but also RCVS. Endothelial damage might be an underlining cause and vasospasm after delivery is a trigger of RCVS. Plasma exchange was effective against both TTP and RCVS.

Original languageEnglish
Pages (from-to)790-792
Number of pages3
JournalTransfusion and Apheresis Science
Volume57
Issue number6
DOIs
Publication statusPublished - Dec 2018

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Thrombotic Thrombocytopenic Purpura
Vasoconstriction
Pregnancy
Plasma Exchange
Cerebral Arteries
Thrombocytopenia
Pathologic Constriction
Primary Headache Disorders
Cerebral Infarction
Postpartum Period
Anemia
Siblings

All Science Journal Classification (ASJC) codes

  • Hematology

Cite this

Upshaw-Schulman syndrome diagnosed during pregnancy complicated by reversible cerebral vasoconstriction syndrome. / Tsuda, Mariko; Shiratsuchi, Motoaki; Nakashima, Yasuhiro; Ikeda, Motohiko; Muta, Hiroki; Narazaki, Taisuke; Masuda, Toru; Kimura, Daisaku; Takamatsu, Akiko; Matsumoto, Masanori; Fujimura, Yoshihiro; Kokame, Koichi; Matsushima, Takamitsu; Ogawa, Yoshihiro.

In: Transfusion and Apheresis Science, Vol. 57, No. 6, 12.2018, p. 790-792.

Research output: Contribution to journalArticle

Tsuda, M, Shiratsuchi, M, Nakashima, Y, Ikeda, M, Muta, H, Narazaki, T, Masuda, T, Kimura, D, Takamatsu, A, Matsumoto, M, Fujimura, Y, Kokame, K, Matsushima, T & Ogawa, Y 2018, 'Upshaw-Schulman syndrome diagnosed during pregnancy complicated by reversible cerebral vasoconstriction syndrome', Transfusion and Apheresis Science, vol. 57, no. 6, pp. 790-792. https://doi.org/10.1016/j.transci.2018.10.023
Tsuda, Mariko ; Shiratsuchi, Motoaki ; Nakashima, Yasuhiro ; Ikeda, Motohiko ; Muta, Hiroki ; Narazaki, Taisuke ; Masuda, Toru ; Kimura, Daisaku ; Takamatsu, Akiko ; Matsumoto, Masanori ; Fujimura, Yoshihiro ; Kokame, Koichi ; Matsushima, Takamitsu ; Ogawa, Yoshihiro. / Upshaw-Schulman syndrome diagnosed during pregnancy complicated by reversible cerebral vasoconstriction syndrome. In: Transfusion and Apheresis Science. 2018 ; Vol. 57, No. 6. pp. 790-792.
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