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Fourteen patients with PTA deficiency are reviewed, including a family with six bleeders in the past two generations. Data are presented which show that the bleeding tendency in this disorder is often mild, as hitherto emphasized, but may also be of moderate or severe degree. Heredity appears to be by an autosomal dominant gene with variable expressivity and incomplete penetrance.

Rosenthal, Dreskin and Rosenthal (1953) described a familial haemorrhagic disorder due to plasma thromboplastin antecedent (PTA) deficiency. Further studies have established the properties of this factor and its action in the early phase of thromboplastin generation (Rosenthal, 1954; Rosenthal, Dreskin and Rosenthal, 1955; Ramot, Angelopoulos and Singer, 1955; Campbell, Mednikoff and Dameshek, 1957; Cavins and Wall, 1960). The mode of hereditary transmission of this deficiency has been discussed by various authors (Cavins and Wall, 1960; Campbell et al., 1957; Rosenthal et al., 1955). Recently Rapaport, Proctor, Patch and Yettra (1961), using a quantitative assay of PTA, suggested that this deficiency is transmitted by an intermediate gene, which produces major PTA deficiency in the homozygote and minor PTA deficiency in the heterozygote. In the present communication, 10 families with PTA deficiency will be described and the mode of its inheritance will be discussed.

The preoperative preparation of patients manifesting a systemic hemorrhagic diathesis has always presented the physician with a serious and often difficult problem. Very few reports dealing with the correction of the coagulation defect of plasma thromboplastin antecedent (PTA) deficiency are to be found in the literature. Rosenthal et al. have been able to correct the PTA deficiency of some patients with a single transfusion of 500 cc. of seven-day-old plasma. 1 , 2 The correction, as measured by in vitro techniques, persisted for seven days in one patient. Another patient was normalized for 24 hours after infusion of only 50 cc. of plasma. 3 The patient reported in this article required transfusions of large amounts of blood for correction of the in vitro test. This patient was previously reported from this department as a well-documented case of PTA deficiency. 4 Methods The routine hematologic tests were performed with the usual methods.

Summary Two cases of previously undiagnosed plasma thromboplastin antecedent deficiency are reported. The problems associated with the diagnosis and management of this condition are discussed. Recommendations are made for the control of this deficiency in routine cases.

Until recently, male persons with a severe hemorrhagic diathesis characterized by (1) a prolonged clotting time, (2) normal Quick plasma prothrombin time, but (3) a poor prothrombin consumption were unhesitatingly diagnosed as suffering from classic hemophilia. This disease is still widely attributed to a deficiency of antihemophilic factor (AHF) 1 essential for the formation of blood thromboplastin. However, it has been emphasized that the demonstrable decrease of AHF activity in these patients may be functional rather than real, owing to the presence of an inhibitor (antithromboplastin). 2 In 1952, Aggeler and co-workers 3 described a syndrome, clinically and genetically indistinguishable from classic hemophilia, which is apparently caused by the absence or marked diminution of a previously unrecognized plasma constituent. They named this factor plasma thromboplastin component (PTC). PTC is also essential for the formation of blood thromboplastin. The PTCdeficiency syndrome is now known under a variety of names, such as

Es wird uber eine Sonderform der Hamophilie bei 2 Brudern berichtet, die sich klinisch nicht von der klassischen Hamophilie unterschied. In beiden Fallen handelt es sich um Mangel an einem Plasmafaktor, der nicht an Bariumsulfat adsorbiert wird und auch im Serum erhalten bleibt. Dieses Verhalten entspricht der vonRosenthal beschriebenen PTA-Mangel-Hamophilie.

This is an unusually complete and enlightened discussion of hemorrhagic states in terms which will really help the practicing physician in his understanding and treatment of these problems. Emphasis is placed on bleeding states associated with hereditary deficiencies of clotting factors and includes such recently described coagulation defects as plasma thromboplastin antecedent deficiency and Hageman trait. Hageman trait is of special interest in that it is characterized by lack of a clotting activity easily demonstrated in the laboratory, but is unassociated with a bleeding tendency. In all these conditions the patterns of inheritance are reviewed together with the problems of daily living which face a family fated to one of these conditions. The thrombocytopenias are considered very logically. Discussion extends to such conditions as scurvy, purpura with dysproteinemia, autoerythrocyte sensitization, and also the hemorrhagic complications of extracorporeal circulation. Items of importance in making a clinical diagnosis are covered, which include

An increasing number of hereditary hemorrhagic diseases caused by deficiencies in plasma clotting factors has been recognized in recent years. In addition to classic hemophilia or antihemophilic globulin deficiency, two other similar diseases have been described: plasma-thromboplastin-component deficiency, or Christmas disease, 1 and plasma-thromboplastin-antecedent deficiency. 2 In comparison with antihemophelic-globulin and plasma-thromboplastin-componentdeficiencies, hemorrhagic manifestations have been generally less severe in plasma-thromboplastin-antecedent deficiency. Spontaneous hemorrhage has been rare. This paper presents an unusual case of plasmathromboplastin-antecedent deficiency in which spontaneous cerebral hemorrhage produced bizarre neurological symptoms. The prompt disappearance of symptoms after the administration of plasma emphasized the importance of establishing the diagnosis of plasma-thromboplastin-antecedent deficiency. A 38-year-old housewife had an extensive history of abnormal bleeding. At the age of 17, she had had severe postpartum bleeding. At the age of 28, she required five blood transfusions for hemorrhage after mastoid surgery. Three years before the present studies, she bled