Prekallikrein deficiency

Area of Science:

• Hematology
• Coagulation Disorders
• Clinical Nursing

Background:

• Abnormal laboratory assays necessitate further investigation for optimal patient care.
• A prolonged activated partial thromboplastin time (PTT) with a normal prothrombin time (PT) may indicate laboratory error or underlying bleeding disorders.
• Prekallikrein deficiency, a rare autosomal recessive coagulation disorder, is characterized by a prolonged PTT and normal PT.

Purpose of the Study:

• To provide nurses with a foundational understanding of hemostasis.
• To identify six potential causes for a prolonged PTT with a normal or slightly prolonged PT.
• To present two novel cases of recently diagnosed adult prekallikrein deficiency.

Main Methods:

• Literature review on hemostasis and coagulation disorders.
• Analysis of diagnostic criteria for prolonged PTT and normal PT.
• Case study presentation of two adult patients with prekallikrein deficiency.

Main Results:

• Prekallikrein deficiency, historically known as Fletcher factor deficiency, presents with a prolonged PTT and normal PT.
• Six distinct causes for this laboratory finding were identified.
• Two previously unreported adult cases of prekallikrein deficiency were diagnosed.

Conclusions:

• Nurses play a crucial role in recognizing and investigating abnormal coagulation assays.
• Understanding rare bleeding disorders like prekallikrein deficiency is essential for accurate diagnosis and patient management.
• This review equips nurses with knowledge to differentiate prekallikrein deficiency from other causes of prolonged PTT.