title: [Early diagnosis and successful treatment of catastrophic antiphospholipid syndrome complicated by multiple organ failure].
abstract: A 50-year-old woman was emergently admitted because of rapidly progressive unconsciousness, renal failure, hepatic dysfunction, hemolytic anemia, thrombocytopenia, and high-grade fever in July, 2008. Based on clinical and laboratory findings, we made a tentative diagnosis of thrombotic thrombocytopenic purpura (TTP) and immediately initiated the plasma exchange (PE). Despite the PE, she developed panperitonitis due to multiple intestinal perforation and massive splenic infarction within a week after the admission. Thrombosis of arterioles at perforated portion in the resected small and large intestines was histologically confirmed. Therefore, we made a definite diagnosis of catastrophic antiphospholipid syndrome (CAPS) based on the presence of antiphosphatidylserine-prothrombin complex antibodies (aPS/PT) throughout the course and lupus anticoagulant that was revealed positive on one occasion, and multiple thrombotic lesions. The underlying disease of CAPS appeared to be lupus erythematosus because of her clinical history and laboratory findings such as persistent leukopenia. Although it has been reported that CAPS causes systemic thrombosis at microvessels mostly within a week from the onset and the mortality rate in this disorder is as high as 50%, we successfully treated her in combination with high-dose corticosteroid, anticoagulation, concentrated human IgG, surgical procedures, and hemodialysis in addition to PE. Early diagnosis of CAPS and immediate start of PE may have contributed to the successful treatment.
authors.affiliation: Department of Hematology and Clinical Immunology, Kobe City Medical Center General Hospital, Kobe, Japan.
authors.forename: Daichi
authors.initials: D
authors.lastname: Inoue
authors.forename: Katsuhiro
authors.initials: K
authors.lastname: Togami
authors.forename: Norihiro
authors.initials: N
authors.lastname: Shimoike
authors.forename: Ryo
authors.initials: R
authors.lastname: Tamura
authors.forename: Yukihiro
authors.initials: Y
authors.lastname: Imai
authors.forename: Takaharu
authors.initials: T
authors.lastname: Kimura
authors.forename: Sonoko
authors.initials: S
authors.lastname: Shimoji
authors.forename: Minako
authors.initials: M
authors.lastname: Mori
authors.forename: Yuya
authors.initials: Y
authors.lastname: Nagai
authors.forename: Sumie
authors.initials: S
authors.lastname: Tabata
authors.forename: Akiko
authors.initials: A
authors.lastname: Matsushita
authors.forename: Kenichi
authors.initials: K
authors.lastname: Nagai
authors.forename: Takayuki
authors.initials: T
authors.lastname: Takahashi
mesh_terms: D016736:Antiphospholipid Syndrome / Q000175:diagnosis* / Q000628:therapy*; D005260:Female; D006801:Humans; D008875:Middle Aged; D009102:Multiple Organ Failure / Q000209:etiology*