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Zorluk: OrtaHematology and Oncology

Match each patient's clinical presentation and laboratory coagulation profile to the most likely underlying hemostatic disorder.

  • A 24-year-old woman with menorrhagia and epistaxis; laboratory studies show a normal PT, prolonged aPTT, and prolonged bleeding time.von Willebrand disease
  • A 6-year-old boy with a swollen, painful knee after a minor fall; laboratory studies show a normal PT, prolonged aPTT, and normal bleeding time. A mixing study with normal plasma fully corrects the aPTT.Hemophilia A
  • A 65-year-old man with unprovoked, extensive soft tissue bleeding; laboratory studies show a normal PT, prolonged aPTT, and normal bleeding time. A mixing study with normal plasma fails to correct the aPTT.Acquired factor VIII inhibitor
  • A 32-year-old woman with placental abruption who develops continuous oozing from venipuncture sites; laboratory studies show prolonged PT, prolonged aPTT, thrombocytopenia, and low fibrinogen.Disseminated intravascular coagulation (DIC)

Cevap

The correct matches correlate von Willebrand disease with combined primary and secondary hemostasis defects, Hemophilia A with a correctable prolonged aPTT, acquired factor VIII inhibitor with an uncorrectable prolonged aPTT, and DIC with consumptive coagulopathy.
Each clinical scenario presents a distinct coagulopathy. von Willebrand disease affects both primary hemostasis and factor VIII stability. Hemophilia represents a true factor deficiency that corrects with normal plasma. Acquired inhibitors neutralize coagulation factors, resisting correction in mixing studies. DIC causes global depletion of coagulation factors, fibrinogen, and platelets.

Adım Adım Çözüm

1
Analyze the bleeding pattern.
Mucocutaneous bleeding (epistaxis, menorrhagia) suggests a primary hemostatic defect, whereas deep tissue bleeding (hemarthrosis, large hematomas) suggests a secondary hemostatic (coagulation factor) defect.
Bleeding phenotype helps initially categorize the disorder.
2
Interpret the PT and aPTT.
An isolated prolonged aPTT points to the intrinsic pathway (factors VIII, IX, XI, XII). Prolongation of both PT and aPTT indicates a common pathway defect or consumptive process.
PT evaluates the extrinsic pathway, while aPTT evaluates the intrinsic pathway.
3
Evaluate the results of the mixing study for isolated prolonged aPTT.
Correction implies a factor deficiency, while failure to correct implies the presence of an inhibitor (such as an autoantibody).
Mixing studies differentiate between missing factors and neutralizing antibodies.

Anahtar Kavram

Interpretation of coagulation panels (PT, aPTT, bleeding time) and mixing studies to differentiate hemostatic disorders.
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