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Difficulty: MediumHematology and Oncology

For each distinct laboratory and clinical profile in patients presenting with bleeding symptoms, match the most accurate underlying diagnosis.

  • Prolonged activated partial thromboplastin time (aPTT) that corrects with a mixing study, normal prothrombin time (PT), and normal platelet count in a male with recurrent hemarthrosis.Factor VIII deficiency
  • Prolonged PT, prolonged aPTT, decreased fibrinogen, elevated D-dimer, and thrombocytopenia in a patient with gram-negative bacteremia.Disseminated intravascular coagulation
  • Isolated prolonged PT, normal aPTT, and normal platelet count in a patient with a history of recurrent mild epistaxis.Factor VII deficiency
  • Prolonged bleeding time, prolonged aPTT, normal PT, and normal platelet count in a female with heavy menstrual bleeding.von Willebrand disease

Answer

The correct matches logically connect specific patterns of intrinsic, extrinsic, and common pathway coagulation defects with their classic hematologic disorders.
Each coagulation profile maps directly to a specific physiological part of the hemostatic pathway. Isolated correctable aPTT prolongation implies an intrinsic factor deficiency. Global cascade prolongation with thrombocytopenia indicates consumptive coagulopathy. Isolated PT prolongation zeroes in on the extrinsic pathway. Combined aPTT and bleeding time prolongation indicates concurrent primary and secondary hemostatic defects.

Step-by-Step Solution

1
Analyze the first profile showing isolated aPTT prolongation that corrects with a mixing study.
Points to an intrinsic pathway factor deficiency (Hemophilia).
A mixing study correction rules out autoantibodies (inhibitors), identifying a pure factor deficiency in the intrinsic cascade (Factor VIII, IX, XI, or XII).
2
Evaluate the second profile demonstrating global coagulation failure with thrombocytopenia.
Corresponds to a consumptive coagulopathy (DIC).
Sepsis triggers widespread tissue factor release, simultaneously consuming clotting factors and platelets while activating fibrinolysis.
3
Examine the third profile characterized by an isolated PT prolongation.
Indicates an isolated extrinsic pathway defect.
Factor VII is uniquely responsible for the extrinsic pathway; an isolated deficiency will only prolong the PT without affecting the aPTT or bleeding time.
4
Assess the fourth profile featuring combined primary (bleeding time) and secondary (aPTT) hemostasis defects.
Identifies von Willebrand disease.
vWF mediates initial platelet adhesion (primary hemostasis) and stabilizes Factor VIII in the plasma (secondary hemostasis), leading to this dual laboratory abnormality.

Key Concept

Interpretation of coagulation panels and platelet counts to diagnose inherited and acquired bleeding disorders.
Estimated Time:1m 30s
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Topics

Hematology and Oncology

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