Plasma Thromboplastin Antecedent Deficiency Research Articles

Roles of factor XI, platelets and tissue factor-initiated blood coagulation

Roles of factor XI, platelets and tissue factor-initiated blood coagulation

Factor XI Deficiency–Related Spontaneous Primary Intraventricular Hemorrhage

Factor XI deficiency (plasma thromboplastin antecedent deficiency) is a rare autosomal-dominant disorder. Neurologic complications in factor XI deficiency are even rarer. We propose that the factor XI deficiency in our patient contributed to a primary intraventricular hemorrhage.

Factor XI Deficiency-Related Spontaneous Primary Intraventricular Hemorrhage

Factor XI deficiency (plasma thromboplastin antecedent deficiency) is a rare autosomal-dominant disorder. Neurologic complications in factor XI deficiency are even rarer. We propose that the factor XI deficiency in our patient contributed to a primary intraventricular hemorrhage.

Plasma Thromboplastin Antecedent (Factor XI) Deficiency in a Black Family

A black man with a prolonged partial thromboplastin time has a severe deficiency of plasma thromboplastin antecedent (PTA) (factor XI) measured both in clotting assays and immunoassays, suggesting a diagnosis of homozygous PTA deficiency. His offspring seemed to be heterozygous carriers of PTA deficiency. Additionally, the proband and two of his children had decreased Hageman factor (factor XII) levels consistent with those of heterozygous carriers of Hageman trait. To our knowledge, this is the first case known of PTA deficiency in a black person. Its pattern of inheritance was independent of that of factor VII deficiency.

Ophthalmic Surgery and Plasma Thromboplastin Antecedent (Factor XI) Deficiency

A patient had bilateral cataracts and severe plasma thromboplastin antecedent deficiency (factor XI deficiency). This patient underwent successful cataract extraction in both eyes without complication. The problems inherent in performing eye surgery with factor XI deficiency are discussed.

Factor XI Deficiency-Reply

In Reply.— Dr Soloway presents a number of pertinent questions and comments, certain of which were spoken to in the original editorially scrutinized manuscript, later contracted for publication. Answers to his specific questions are as follows: (1) 45 seconds; (2) kaolin; (3) no, specific factor analyses were performed, and only factor XI was below the generally accepted norms for the several factors; (4) no; and (5) no. In 1953, Rosenthal described a type of hemophilia owing to plasma thromboplastin antecedent deficiency (PTA), or hemophilia C.1Following elucidation of this defect, other cases were reported in the literature. Two types of patients were defined. The first group was severely affected with multiple episodes of bleeding and hemorrhage; a second group was much less symptomatic, often only identifiable by clotting factor analysis. The severely affected group on clotting factor analysis was noted to have a factor XI level of 20% of

7a] Bovine factor XI (plasma thromboplastin antecedent)

Factor XI is a plasma glycoprotein that participates in the early stage of intrinsic blood coagulation. It is absent, or present as an inert protein, in plasma of individuals with a congenital bleeding disorder called plasma thromboplastin antecedent deficiency. In normal plasma, factor XI is present in a precursor form and is converted to an active form (factor XIa) by another coagulation factor(s). The purification of factor XI has been difficult by conventional methods of protein fractionation since its concentration in plasma is low and it is also very sensitive to protease degradation. This chapter describes the isolation method in which, heparin-agarose column chromatography and benzamidine–agarose column chromatography play an important role in the procedure. It also details each purification step which is performed in the presence of several protease inhibitors, making it possible to isolate a highly purified preparation in a precursor form. It describes the purification process and the general properties of Factor XI.

Studies on plasma thromboplastin antecedent (factor XI), PTA deficiency and inhibition of PTA by plasma: pharmacologic inhibitors and specific antiserum.

Abstract Partially purified, activated PTA (plasma thromboplastin antecedent) (factor XI), prepared by a method similar to that used earlier, had an apparent molecular weight of 165,000 and a sedimentation constant of 8S. It was inhibited by normal plasma, phenylmethyl sulfonylfluoride, and by agents in crude potato ATPase and acid phosphatase preparations. An antiserum was prepared against activated PTA which inactivated this factor in normal human plasma. Immunologic studies demonstrated that the plasma of patients with hereditary PTA deficiency was deficient in antigens related to this factor, suggesting that in this disorder, synthesis of PTA is impaired. The plasma of patients with PTA deficiency possessed normal inhibitory activity against activated PTA.

Plasma thromboplastin antecedent deficiency

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.

Heredity and coagulation studies in ten families with Factor XI (plasma thromboplastin antecedent) deficiency.

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.

A Clinical Evaluation of Plasma Thromboplastin Antecedent (PTA) Deficiency

Article1 May 1965A Clinical Evaluation of Plasma Thromboplastin Antecedent (PTA) DeficiencyFRED G. CONRAD, WILLIAM L. BRENEMAN, DOROTHY B. GRISHAM, B.S.FRED G. CONRADSearch for more papers by this author, WILLIAM L. BRENEMANSearch for more papers by this author, DOROTHY B. GRISHAM, B.S.Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-62-5-885 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptSince Rosenthal, Dreskin, and Rosenthal (1) first described plasma thromboplastin antecedent (PTA) deficiency 10 years ago, investigators from numerous hematology centers have emphasized the infrequent occurrence of this defect. Reports of the relative rate of occurrence of patients deficient in antihemophilic factor (AHF) and plasma thromboplastin component (PTC) are remarkably stable at a 4 to 1 ratio in this country and throughout the world (2-4). The reports of relative occurrence of PTA show marked variations from less than 1% to 18% of the total thromboplastinogen disorders (5-8).Contrary to the usually reported low incidence, our studies indicate that PTA deficiency...

Prolonged bleeding time in a patient withplasma thromboplastin antecedent deficiency: Observations on correction of the bleeding time by platelet transfusion

A 10-year-old child with a family and personal history suggestive of a bleedingdiathesis was found to have a prolonged bleeding time in addition to a defect in the first stage of coagulation due to plasma thromboplastin antecedent deficiency. Further studies revealed a defect in the in vivo adhesiveness of his platelets. In order to ascertain the basis for the prolonged bleeding time, the patient received a transfusion of fresh plasma and platelets. Platelet transfusion corrected the prolonged bleeding time and abnormal in vivo platelet adhesion, whereas the plasma had little effect. These results demonstrate the value of transfusion in elucidating the etiology of obscure bleeding disorders.

Assay of Plasma Thromboplastin Antecedent (PTA) with Artificially Depleted Normal Plasma

Abstract Plasma artificially depleted of PTA by incubation following activation with diatomaceous silica was evaluated as a reagent for the assay of PTA. It gave results which were comparable to those obtained with naturally deficient plasma and had the advantages of being readily available and more stable on storage. Laboratory differentiation was possible between PTA and HF deficiency.

Bleeding Syndromes: A Clinical Manual

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

Clinical and laboratory studies of plasma thromboplastin antecedent deficiency (PTA)

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.

The correction of a hemorrhagic diathesis in preparation for surgery; the correction of plasma thromboplastin antecedent deficiency.

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.

Plasma Thromboplastin Antecedent (PTA) Deficiency: Clinical, Coagulation, Therapeutic and Hereditary Aspects of a New Hemophilia-like Disease

Abstract 1. An analysis of the original PTA deficient family, including coagulation studies performed upon 13 members comprising 4 generations, has been presented. 2. PTA deficiency is transmitted as an autosomal dominant trait with a probable high degree of penetrance and variable expression of the gene. 3. PTA deficiency can occur its varying degrees ranging from a severe form with prolonged clotting time and markedly abnormal heparin clotting time and prothrombin utilization to a mild form manifesting a normal clotting time and slightly impaired prothrombin utiliztition. 4. Studies on the treatment of PTA deficiency reveal that the defect is corrected by the administration of stored plasma with the effect gradually disappearing over the period of one week. 5. Various properties of PTA are discussed and compared with AHG and PTC.