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Prekallikrein deficiency: the characteristic normalization of the severely prolonged aPTT following increased preincubation time is due to autoactivation of factor XII.
Thromb Res
March 2002
Central Hematology Laboratory, University of Bern, Inselspital, Bern CH 3010, Switzerland.
Hereditary plasma prekallikrein (PK) deficiency was diagnosed in a 71-year-old man with an 8-year history of osteomyelofibrosis. PK deficiency was suspected in view of a severely prolonged activated partial thromboplastin time (aPTT) that nearly normalized following prolonged preincubation (10 min) of patient plasma with kaolin-inosithin reagent. Hereditary PK deficiency was demonstrated by very low PK values in the propositus (PK clotting activity 5%, PK amidolytic activity 5%, PK antigen 2% of normal plasma, respectively) and half normal PK values in his children.
View Article and Find Full Text PDFThe Passovoy defect is a recently characterized hemorrhagic diathesis. We describe a patient with a febrile illness, possibly from Epstein-Barr (EB) virus, who acquired this defect transiently. Prothrombin time; assays for factors VIII, IX, XI, XII; and Fletcher (prekallikrein) and Fitzgerald (high molecular weight kininogen) factors were normal.
View Article and Find Full Text PDFPassovoy factor deficiency, a coagulation abnormality affecting the intrinsic coagulation system, was discovered in 5 Western Australian kindreds. The defect is inherited as an autosomal dominant and is associated with a clinical bleeding tendency characterized by easy bruising and undue blood loss following trauma such as dental extraction and tonsillectomy. Fresh frozen plasma appears to provide effective prophylaxis during surgery.
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