Diagnostic Pearls and Clinical Implications of Prekallikrein Deficiency

Abstract

Prekallikrein (PK) deficiency is extremely rare, and manifestations are not well characterized due to a small number of cases reported and the lack of scientific clarity about its role in clot formation in vivo. Here, we report a case of a 64-year-old male, with no known history of abnormal bleeding, who scheduled to undergo deep brain stimulator placement for control of his Parkinson's disease. During pre-procedure testing, activated partial thromboplastin time (PTT) was found to be prolonged at 146 seconds. Mixing studies were suggestive of a coagulation factor deficiency. His PTT characteristically became shorter with prolonged incubation, providing a clue at testing for PK levels, which were found to be severely low. He, subsequently, underwent surgery without any complications. Our case further highlights the clinical pearls for diagnosis and further endorses that these patients can safely undergo surgical procedures without the need for plasma transfusions or factor concentrate usage.

Abnormal ACT in a Patient with Prekallikrein Deficiency Undergoing Cardiopulmonary Bypass

“…Congenital Prekallikrein Open Journal of Anesthesiology recessive defect usually diagnosed incidentally during routine coagulation tests demonstrating substantially prolonged activated Partial Thromboplastin Time (aPTT) and normal Prothrombin Time (PT) without associated bleeding abnormalities [3]. Since this condition is rare, and exact prevalence is possibly unknown as it is not associated with bleeding, thrombosis or any other severe clinical symptoms [4], thus, the characterization of its phenotype is not well elucidated. Here, we report anesthesia management of a pediatric patient with PK deficiency on cardiopulmonary bypass with abnormal ACT, but does not demonstrate abnormal coagulation issues.…”

False Prolongation of Activated Partial Thromboplastin Time with Aminoglycoside Antimicrobial Agents: A Case Report

“…Prolonged APTT indicates decreased or inactive coagulation factors, von Willebrand factor [7], prekallikrein [8], decreased activity of high molecular weight kininogen [9], and the presence of a lupus anticoagulant (LA) [10], [11]. Specific diseases and conditions include von Willebrand disease, hemophilia, disseminated intravascular syndrome, and Vitamin K deficiency [12], [13].…”

Prevalence of Cardiovascular Disorders in African-Americans With Congenital Prekallikrein Deficiency Versus Caucasians-Americans With the Same Defect

“…AA with PK deficiency and CVD are gathered in Table 1. [11][12][13][14][15][16][17][18][19][20][21][22][23][24] It has to be noted that for a few papers dealing with PK defects, no ethnic background is indicated. It is likely that at least some of them may also involve AA.…”