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Research Article Free access | 10.1172/JCI109387
Department of Medicine and Institute of Hematology, Jichi Medical School, Tochigi-Ken, Japan 329-04
Department of Medicine, Case Western Reserve University School of Medicine, Cleveland, Ohio 44106
Department of Medicine, Nagoya University School of Medicine, Nagoya, Japan 466
Kobakura Clinic, Okinawa, Japan 900
*An abbreviated version of this report has appeared in 1978: Lancet. II: 1334-1336. Dr. Aoki and Dr. Saito assert that this paper was submitted to Lancet without their knowledge. The Editor.
Find articles by Aoki, N. in: JCI | PubMed | Google Scholar
Department of Medicine and Institute of Hematology, Jichi Medical School, Tochigi-Ken, Japan 329-04
Department of Medicine, Case Western Reserve University School of Medicine, Cleveland, Ohio 44106
Department of Medicine, Nagoya University School of Medicine, Nagoya, Japan 466
Kobakura Clinic, Okinawa, Japan 900
*An abbreviated version of this report has appeared in 1978: Lancet. II: 1334-1336. Dr. Aoki and Dr. Saito assert that this paper was submitted to Lancet without their knowledge. The Editor.
Find articles by Saito, H. in: JCI | PubMed | Google Scholar
Department of Medicine and Institute of Hematology, Jichi Medical School, Tochigi-Ken, Japan 329-04
Department of Medicine, Case Western Reserve University School of Medicine, Cleveland, Ohio 44106
Department of Medicine, Nagoya University School of Medicine, Nagoya, Japan 466
Kobakura Clinic, Okinawa, Japan 900
*An abbreviated version of this report has appeared in 1978: Lancet. II: 1334-1336. Dr. Aoki and Dr. Saito assert that this paper was submitted to Lancet without their knowledge. The Editor.
Find articles by Kamiya, T. in: JCI | PubMed | Google Scholar
Department of Medicine and Institute of Hematology, Jichi Medical School, Tochigi-Ken, Japan 329-04
Department of Medicine, Case Western Reserve University School of Medicine, Cleveland, Ohio 44106
Department of Medicine, Nagoya University School of Medicine, Nagoya, Japan 466
Kobakura Clinic, Okinawa, Japan 900
*An abbreviated version of this report has appeared in 1978: Lancet. II: 1334-1336. Dr. Aoki and Dr. Saito assert that this paper was submitted to Lancet without their knowledge. The Editor.
Find articles by Koie, K. in: JCI | PubMed | Google Scholar
Department of Medicine and Institute of Hematology, Jichi Medical School, Tochigi-Ken, Japan 329-04
Department of Medicine, Case Western Reserve University School of Medicine, Cleveland, Ohio 44106
Department of Medicine, Nagoya University School of Medicine, Nagoya, Japan 466
Kobakura Clinic, Okinawa, Japan 900
*An abbreviated version of this report has appeared in 1978: Lancet. II: 1334-1336. Dr. Aoki and Dr. Saito assert that this paper was submitted to Lancet without their knowledge. The Editor.
Find articles by Sakata, Y. in: JCI | PubMed | Google Scholar
Department of Medicine and Institute of Hematology, Jichi Medical School, Tochigi-Ken, Japan 329-04
Department of Medicine, Case Western Reserve University School of Medicine, Cleveland, Ohio 44106
Department of Medicine, Nagoya University School of Medicine, Nagoya, Japan 466
Kobakura Clinic, Okinawa, Japan 900
*An abbreviated version of this report has appeared in 1978: Lancet. II: 1334-1336. Dr. Aoki and Dr. Saito assert that this paper was submitted to Lancet without their knowledge. The Editor.
Find articles by Kobakura, M. in: JCI | PubMed | Google Scholar
Published May 1, 1979 - More info
α2-Plasmin inhibitor (α2PI) is a recently characterized, fast-reacting plasmin inhibitor in human plasma that appears to play an important role in regulation of in vivo fibrinolysis. We report here a case of complete deficiency of α2PI in man. The patient, a 25-yr-old Japanese man, had a life-long severe bleeding tendency (hemarthrosis and excessive bleeding after trauma). The following tests were within normal limits: platelet count, bleeding time, thrombin time, prothrombin time, partial thromboplastin time, titers of known clotting factors, platelet glass bead retention, Factor VIII-related antigen, platelet aggregation by ADP, collagen and ristocetin, and clot retraction. Routine liver function tests were also normal. The only abnormal finding was that whole blood clot lysis was extemely rapid and was complete in 4-8 h. The concentration of plasma protease inhibitors, including α2-macro-globulin, antithrombin III, α1-antitrypsin, and C1̄INH, were all normal. The concentration of α2-PI in the patient's plasma, assayed by immunological methods, was <0.1 mg/100 ml (normal concentration, 6.1±0.88 mg/100 ml [mean±SE]) and functional assays showed a complete deficiency of α2PI. Addition of purified α2PI to the patient's whole blood completely corrected the accelerated fibrinolysis. The patient's parents, four siblings, and four other members of this family were asymptomatic, but the titers of α2PI in their plasmas were ≅50% of normal pooled plasma. There were three consanguineous marriages in this family, and the α2PI deficiency appears to have been inherited as an autosomal recessive trait. We speculate that α2PI deficiency in this patient has led to uninhibited in vivo fibrinolysis that probably causes the severe hemorrhagic tendency. Thus, this study indicates the important role of α2PI in hemostasis.
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