Upshaw-Schulman syndrome diagnosed during pregnancy complicated by reversible cerebral vasoconstriction syndrome.
Transfus Apher Sci
; 57(6): 790-792, 2018 Dec.
Article
en En
| MEDLINE
| ID: mdl-30471945
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.
Palabras clave
Texto completo:
1
Base de datos:
MEDLINE
Asunto principal:
Complicaciones del Embarazo
/
Púrpura Trombocitopénica Trombótica
/
Vasoconstricción
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Cerebro
Tipo de estudio:
Diagnostic_studies
Idioma:
En
Revista:
Transfus Apher Sci
Asunto de la revista:
HEMATOLOGIA
Año:
2018
Tipo del documento:
Article