Acquired factor XI inhibitors in congenitally deficient patients. 1987

S F Schnall, and T P Duffy, and L P Clyne
Department of Medicine, Yale University School of Medicine, New Haven, Connecticut.

Four factor XI (F XI)-deficient patients are described, all of whom formed circulating anticoagulants against F X1. In the three most severely affected patients (F XI 0%-6% activity), the anticoagulant appeared to have been stimulated by plasma infusion. However, in the milder case (25% F XI activity), no infusion had been documented. The findings in these cases emphasize the diversity of F XI inhibitors in congenitally deficient patients. Awareness of the potential development of these inhibitors will be helpful in both daily management and perioperative care of such patients.

UI MeSH Term Description Entries
D008297 Male Males
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D010314 Partial Thromboplastin Time The time required for the appearance of FIBRIN strands following the mixing of PLASMA with phospholipid platelet substitute (e.g., crude cephalins, soybean phosphatides). It is a test of the intrinsic pathway (factors VIII, IX, XI, and XII) and the common pathway (fibrinogen, prothrombin, factors V and X) of BLOOD COAGULATION. It is used as a screening test and to monitor HEPARIN therapy. Activated Partial Thromboplastin Time,Cephalin-Kaolin Coagulation Time,Kaolin-Cephalin Coagulation Time,Thromboplastin Time, Partial,Coagulation Time, Cephalin-Kaolin,Cephalin Kaolin Coagulation Time,Coagulation Time, Cephalin Kaolin,Coagulation Time, Kaolin-Cephalin,Kaolin Cephalin Coagulation Time
D010375 Pedigree The record of descent or ancestry, particularly of a particular condition or trait, indicating individual family members, their relationships, and their status with respect to the trait or condition. Family Tree,Genealogical Tree,Genealogic Tree,Genetic Identity,Identity, Genetic,Family Trees,Genealogic Trees,Genealogical Trees,Genetic Identities,Identities, Genetic,Tree, Family,Tree, Genealogic,Tree, Genealogical,Trees, Family,Trees, Genealogic,Trees, Genealogical
D011517 Prothrombin Time Clotting time of PLASMA recalcified in the presence of excess TISSUE THROMBOPLASTIN. Factors measured are FIBRINOGEN; PROTHROMBIN; FACTOR V; FACTOR VII; and FACTOR X. It is used for monitoring anticoagulant therapy with COUMARINS. Quick Test,Russell's Viper Venom Time,Thrombotest,Russell Viper Venom Time,Russells Viper Venom Time,Prothrombin Times,Test, Quick,Time, Prothrombin,Times, Prothrombin
D005172 Factor XI Stable blood coagulation factor involved in the intrinsic pathway. The activated form XIa activates factor IX to IXa. Deficiency of factor XI is often called hemophilia C. Coagulation Factor XI,Plasma Thromboplastin Antecedent,Blood Coagulation Factor XI,Factor 11,Factor Eleven,Antecedent, Plasma Thromboplastin,Factor XI, Coagulation,Thromboplastin Antecedent, Plasma
D005173 Factor XI Deficiency A hereditary deficiency of blood coagulation factor XI (also known as plasma thromboplastin antecedent or PTA or antihemophilic factor C) resulting in a systemic blood-clotting defect called hemophilia C or Rosenthal's syndrome, that may resemble classical hemophilia. Hemophilia C,Rosenthal Syndrome,Deficiency, Factor 11,Deficiency, Factor Eleven,Deficiency, Factor XI,Factor 11 Deficiency,Factor Eleven Deficiency,Plasma Thromboplastin Antecedent Deficiency,Rosenthal's Syndrome,Deficiencies, Factor 11,Deficiencies, Factor Eleven,Deficiencies, Factor XI,Factor 11 Deficiencies,Factor Eleven Deficiencies,Factor XI Deficiencies,Rosenthal Syndromes,Rosenthal's Syndromes,Rosenthals Syndrome,Syndrome, Rosenthal,Syndrome, Rosenthal's
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D000368 Aged A person 65 years of age or older. For a person older than 79 years, AGED, 80 AND OVER is available. Elderly

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