Table 1.

Bleeding Episodes in Individuals With Homozygous PAI-1 Deficiency

IndividualGenderAgeFamilyBleeding Episodes
Female 15 y Life-threatening postoperative hemorrhage requiring transfusion. Large palatal hemorrhage requiring hospitalization and transfusion. Protracted menstrual bleeding resulting in anemia. 
(Proband)     
Male 4 mo Large epidural hematoma requiring surgical drainage after minor head trauma. 
Female 14 y Large subperiosteal mandibular bleed after striking jaw. Subcutaneous hematomas after minimal trauma. Excessive bruising. 
Male 13 y Elbow and knee hemarthroses after minor trauma. Delayed bleeding after repair of inguinal hernia. 
Female 11 y Frequent bruising. 
Female 5 y Knee hemarthroses. 
Female 2 y Frequent bruising. 
IndividualGenderAgeFamilyBleeding Episodes
Female 15 y Life-threatening postoperative hemorrhage requiring transfusion. Large palatal hemorrhage requiring hospitalization and transfusion. Protracted menstrual bleeding resulting in anemia. 
(Proband)     
Male 4 mo Large epidural hematoma requiring surgical drainage after minor head trauma. 
Female 14 y Large subperiosteal mandibular bleed after striking jaw. Subcutaneous hematomas after minimal trauma. Excessive bruising. 
Male 13 y Elbow and knee hemarthroses after minor trauma. Delayed bleeding after repair of inguinal hernia. 
Female 11 y Frequent bruising. 
Female 5 y Knee hemarthroses. 
Female 2 y Frequent bruising. 

A total of three life-threatening bleeds (in individuals 1 and 2) were observed during 60 patient-years of observation. Family 1 contains the proband, as shown in Fig 1. Family 2 is the family shown in Fig 2.

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