{
    "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.",
    "authors": [
        {
            "affiliation": "Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.",
            "firstname": "Mariko",
            "initials": "M",
            "lastname": "Tsuda"
        },
        {
            "affiliation": "Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan. Electronic address: mshira@intmed3.med.kyushu-u.ac.jp.",
            "firstname": "Motoaki",
            "initials": "M",
            "lastname": "Shiratsuchi"
        },
        {
            "affiliation": "Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.",
            "firstname": "Yasuhiro",
            "initials": "Y",
            "lastname": "Nakashima"
        },
        {
            "affiliation": "Department of Internal Medicine, Nakatsu Municipal Hospital, Nakatsu, Japan.",
            "firstname": "Motohiko",
            "initials": "M",
            "lastname": "Ikeda"
        },
        {
            "affiliation": "Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.",
            "firstname": "Hiroki",
            "initials": "H",
            "lastname": "Muta"
        },
        {
            "affiliation": "Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.",
            "firstname": "Taisuke",
            "initials": "T",
            "lastname": "Narazaki"
        },
        {
            "affiliation": "Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.",
            "firstname": "Toru",
            "initials": "T",
            "lastname": "Masuda"
        },
        {
            "affiliation": "Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.",
            "firstname": "Daisaku",
            "initials": "D",
            "lastname": "Kimura"
        },
        {
            "affiliation": "Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.",
            "firstname": "Akiko",
            "initials": "A",
            "lastname": "Takamatsu"
        },
        {
            "affiliation": "Department of Blood Transfusion Medicine, Nara Medical University, Nara, Japan.",
            "firstname": "Masanori",
            "initials": "M",
            "lastname": "Matsumoto"
        },
        {
            "affiliation": "Japanese Red Cross Kinki Block Blood Center, Ibaraki, Japan.",
            "firstname": "Yoshihiro",
            "initials": "Y",
            "lastname": "Fujimura"
        },
        {
            "affiliation": "Department of Molecular Pathogenesis, National Cerebral and Cardiovascular Center, Suita, Japan.",
            "firstname": "Koichi",
            "initials": "K",
            "lastname": "Kokame"
        },
        {
            "affiliation": "Department of Hematology, Iizuka Hospital, Iizuka, Japan.",
            "firstname": "Takamitsu",
            "initials": "T",
            "lastname": "Matsushima"
        },
        {
            "affiliation": "Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan; Department of Molecular Endocrinology and Metabolism, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Tokyo, Japan.",
            "firstname": "Yoshihiro",
            "initials": "Y",
            "lastname": "Ogawa"
        }
    ],
    "conclusions": null,
    "copyrights": "Copyright \u00a9 2018 Elsevier Ltd. All rights reserved.",
    "doi": "10.1016/j.transci.2018.10.023",
    "journal": "Transfusion and apheresis science : official journal of the World Apheresis Association : official journal of the European Society for Haemapheresis",
    "keywords": [
        "Plasma exchange",
        "Reversible cerebral vasoconstriction syndrome",
        "Thrombotic thrombocytopenic purpura",
        "Upshaw-Schulman syndrome"
    ],
    "methods": null,
    "publication_date": "2018-11-26",
    "pubmed_id": "30471945",
    "results": null,
    "title": "Upshaw-Schulman syndrome diagnosed during pregnancy complicated by reversible cerebral vasoconstriction syndrome.",
    "xml": "<Element 'PubmedArticle' at 0x7885038a1f80>"
}