Factor XI Deficiency: Thrombin and Kallikrein System
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Questions and Answers

A patient with Factor XI deficiency undergoes a minor surgical procedure. Based on the typical clinical presentation of this deficiency, what is the MOST likely bleeding profile they will exhibit?

  • Immediate and profuse bleeding during the procedure that is difficult to control.
  • No bleeding complications observed due to effective pre-operative treatment.
  • Severe, spontaneous bleeding episodes requiring frequent transfusions.
  • Delayed bleeding after the procedure, potentially hours or days later. (correct)

Which of the following laboratory test results would be MOST consistent with a diagnosis of Factor XI deficiency?

  • Normal prothrombin time (PT) with a prolonged activated partial thromboplastin time (aPTT). (correct)
  • Prolonged prothrombin time (PT) with a normal activated partial thromboplastin time (aPTT).
  • Normal prothrombin time (PT) and activated partial thromboplastin time (aPTT).
  • Prolonged prothrombin time (PT) and activated partial thromboplastin time (aPTT).

A 30-year-old woman with known Factor XI deficiency is planning to undergo a hysterectomy. What is the MOST appropriate course of action to manage her bleeding risk?

  • No specific intervention is needed as the procedure is not high-risk.
  • Prescribe oral contraceptives to improve clotting factors.
  • Prophylactic treatment with Factor XI concentrate or plasma before the procedure. (correct)
  • Administer aspirin before and after the procedure.

A researcher is studying the effects of a novel drug on coagulation pathways. They observe that the drug significantly reduces the activity of Factor XI. Which of the following downstream effects would be MOST likely to occur as a direct consequence of this drug?

<p>Impaired activation of Factor IX and reduced amplification of the coagulation cascade. (D)</p> Signup and view all the answers

Which of the following statements BEST describes the inheritance pattern of Factor XI deficiency?

<p>Autosomal recessive, requiring two copies of the affected gene for expression. (D)</p> Signup and view all the answers

Flashcards

Factor XI Deficiency

A deficiency in coagulation factor XI, leading to milder bleeding tendencies compared to other hemophilias.

Bleeding Severity in Factor XI Deficiency

Mild to moderate; often only presenting after surgery or trauma. Spontaneous bleeds are rare

Lab Findings in Factor XI Deficiency

The intrinsic pathway is affected, leading to prolonged aPTT (activated partial thromboplastin time) in lab tests, with aPTT correcting on mixing studies.

Diagnosing Factor XI Deficiency

Diagnosis typically involves measuring factor XI activity level in the blood, which is lower than normal.

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Treatment Options for Factor XI Deficiency

Treatment may include fresh frozen plasma (FFP) or factor XI concentrates before surgery or in case of bleeding. Antifibrinolytic agents can also be considered.

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Study Notes

  • Factor XI Deficiency
  • Authored by Magdalena Dorota Lewandowska, MD, and Jean Marie Connors, MD
  • Published in Hematology/Oncology Clinics, Volume 35, Issue 6, Pages 1157-1169 (December 2021)
  • DOI: 10.1016/j.hoc.2021.07.012

Fig 1

  • Illustrates the structure of Factor XI.

Fig 2

  • Depicts the Thrombin Generation and Kallikrein-Kinin System
  • The Kallikrein-Kinin System includes HKa, BK+AMPs, a-Kal, HK, PK.
  • Thrombin generation includes factors IX, VIIa, TF, Xa and Va.

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Description

Overview of Factor XI deficiency, outlining the structure of Factor XI. Illustration of the Thrombin Generation and Kallikrein-Kinin System, including key components such as HKa, BK+AMPs, and factors IX, VIIa, TF, Xa, and Va.

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