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 language | English |
|---|---|
| Pages (from-to) | 790-792 |
| Number of pages | 3 |
| Journal | Transfusion and Apheresis Science |
| Volume | 57 |
| Issue number | 6 |
| DOIs | |
| Publication status | Published - Dec 2018 |
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All Science Journal Classification (ASJC) codes
- Hematology
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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 journal › Article
}
TY - JOUR
T1 - Upshaw-Schulman syndrome diagnosed during pregnancy complicated by reversible cerebral vasoconstriction syndrome
AU - Tsuda, Mariko
AU - Shiratsuchi, Motoaki
AU - Nakashima, Yasuhiro
AU - Ikeda, Motohiko
AU - Muta, Hiroki
AU - Narazaki, Taisuke
AU - Masuda, Toru
AU - Kimura, Daisaku
AU - Takamatsu, Akiko
AU - Matsumoto, Masanori
AU - Fujimura, Yoshihiro
AU - Kokame, Koichi
AU - Matsushima, Takamitsu
AU - Ogawa, Yoshihiro
PY - 2018/12
Y1 - 2018/12
N2 - 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.
AB - 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.
UR - http://www.scopus.com/inward/record.url?scp=85056872091&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85056872091&partnerID=8YFLogxK
U2 - 10.1016/j.transci.2018.10.023
DO - 10.1016/j.transci.2018.10.023
M3 - Article
C2 - 30471945
AN - SCOPUS:85056872091
VL - 57
SP - 790
EP - 792
JO - Transfusion and Apheresis Science
JF - Transfusion and Apheresis Science
SN - 1473-0502
IS - 6
ER -