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3 young Japanese sisters with congenital α₂-plasmin inhibitor (α₂-PI) deficiency are reported. They have mild umbilical bleeding and/or repeated prolonged bleeding after minor trauma, but rarely spontaneous bleedings. The most characteristic hemostatic findings were shortened whole blood clot lysis time and euglobulin lysis time. Activities of all hemostatic factors except α₂-PI were within normal range. Both functional and immunological absence of α₂-PI were found in the plasma, and this failure to detect α₂-PI was not corrected by the addition of the patient’s plasma of the first described case of α₂-PI deficiency. Clinical and laboratory data revealed that these patients were probably homozygous for α₂-PI deficiency and born of heterozygous parents, but not of consanguineous ones. Bleeding episodes due to deficiency of (α₂-PI in these patients were well controlled by an antifibrinolytic agent, tranexamic acid.

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A 39-year-old man attempted to kill himself using a small knife to penetrate the left anterior chest wall because of trouble at work and with his girlfriend. On arrival at the emergency room, his consciousness was not clear and vital signs were unstable. The knife remained vertically located in the left anterior chest wall. A large left hemothorax was identified by chest X-ray, and moderate cardiac tamponade was detected by echocardiography. Left-sided chest drainage was performed by inserting a chest drainage tube, and about 2500 ml of hemorrhagic effusion was drained. An emergency operation was performed to relieve the cardiac tamponade and repair the penetrating cardiac injury. About an hour after arrival at the emergency room, a median sternotomy was performed in the operating room. The knife had injured the surface of the right ventricular outflow tract, the left lung, and the 3rd intercostal artery and vein. Cardiopulmonary bypass was immediately prepared for the repair of the cardiac injury. The wounds were successfully repaired with pledgeted sutures under cardiac beating. The postoperative course was uneventful with no sign of infection. The patient was discharged at 9 days after the operation. Here we have reported a case of successful surgical repair of a penetrating knife injury to the heart, which was managed by immediate resuscitation and emergency surgery.