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 journalArticlepeer-review

5 Citations (Scopus)


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
Issue number6
Publication statusPublished - Dec 2018

All Science Journal Classification (ASJC) codes

  • Hematology


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