Lege Artis Medicinae

[Activated protein c response: a novel disorder causing thrombophilia]

PÁL András1, SAS Géza1, HAYNAL Imre2, VAJDA Zoltán3, KUNCZ Gabriella3, MOHAMED A. Lateiwish4, RÓNA Tas Ágnes4

NOVEMBER 30, 1994

Lege Artis Medicinae - 1994;4(11)

[In 1993 Dahlbäck described the so-called in vitro activated protein C response phenomena, according to which, the addition of activated protein C to citrated plasma samples prolongs the activated partial thromboplastin times. It was hypothesised that this phenomenon is the result of the action of a new protein C cofactor, and in 1994, Dahlbäck demonstrated that this phenomenon is linked to variations of the factor V, which surprisingly serves as a cofactor for protein C. In this study, forty-one highly selected thrombophilic patients and forty controls were investigated for the activated protein C response phenomenon. The response to activated protein C was expressed as the ratio of two activated partial thromboplastin times, the first in the presence of and the second in the absence of activated protein C. Eighteen patients demonstrated a decreased response, i. e. the ratio of the times was below 2.0 (mean 1.3). The average of the entire group was 1.86. The average ratio of the control group was 3.12, and there were 2 persons with ratios below 2.0. The results of this study correspond with the data in the literature. This newly recognised phenomenon may be responsible for the majority of the formerly unrecognised biochemical defects leading to the clinical picture of thrombophilia. ]

AFFILIATIONS

  1. Hematológiai Tanszék
  2. Egészségtudományi Egyetem
  3. Hemostasis Laboratórium
  4. Hematológiai Osztály Országos Hematológiai Vértranszfúziós és Immunológiai Intézet Budapest

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Lege Artis Medicinae

[The author’s response to the comment on “Exploratory study of outcomes of blood sample mass examinations by rank correlations”]