Welcome to your 8. 🩹 Hemostasis, Platelet Disorders, von Willebrand Disease, Hemophilia & DIC Arena
1.
A 45-year-old man has thrombocytopenia associated with marked splenomegaly. Bone marrow examination shows preserved platelet-producing cells. Which mechanism most likely explains the reduced circulating platelet count?
2.
Which clinical feature is most characteristic of severe hemophilia?
3.
A 28-year-old woman presents with petechiae and menorrhagia. Her platelet count is markedly reduced. Peripheral smear shows large platelets, while hemoglobin and leukocyte counts are preserved. Which finding best explains the marrow response to this disorder?
4.
A patient with mild hemophilia A is being prepared for a minor procedure. Desmopressin is considered appropriate. Which characteristic of the disorder makes this treatment useful?
5.
A 12-year-old boy has recurrent painful swelling of the knee after minor trauma. Platelet count and PT are normal, while aPTT is prolonged. Which disorder is most consistent with these findings?
6.
Which sequence best describes the pathophysiology of disseminated intravascular coagulation?
7.
A 22-year-old woman presents with petechiae and easy bruising. Her platelet count is markedly reduced, while hemoglobin and leukocyte counts are normal. PT and aPTT are also normal. Which diagnosis is most likely?
8.
Hemophilia B results from deficiency of which coagulation factor?
9.
A patient with hemophilia has troublesome bleeding from the oral cavity after a dental procedure. Adequate factor replacement has been given, and an additional drug is prescribed to help preserve the clot at this fibrinolytically active site. Which drug is most appropriate?
10.
Which laboratory finding is most useful as evidence of increased fibrin formation and breakdown in disseminated intravascular coagulation?
11.
A patient with thrombocytopenia has anemia with fragmented red cells on peripheral smear and evidence of small-vessel organ injury. Which mechanism best distinguishes this process from uncomplicated immune thrombocytopenia?
12.
Which finding most strongly supports a diagnosis of uncomplicated immune thrombocytopenia?
13.
A child has severe lifelong mucocutaneous bleeding. Laboratory evaluation shows very little functional von Willebrand factor and markedly reduced factor VIII activity. Which form of von Willebrand disease is most likely?
14.
A woman develops widespread bleeding following a major obstetric complication. Laboratory studies show consumption of platelets and coagulation factors. Which pathological process most directly initiates this pattern?
15.
A patient with immune thrombocytopenia has circulating IgG antibodies directed against platelet membrane glycoproteins. Where are the antibody-coated platelets mainly removed?
16.
Oprelvekin is used to support platelet recovery through which principal action?
17.
Which mechanism is primarily responsible for thrombocytopenia in immune thrombocytopenia?
18.
A boy with an inherited coagulation disorder develops persistent bleeding following a dental procedure. His platelet count is normal, and deficiency of factor IX is confirmed. Which treatment most directly corrects the underlying hemostatic defect?
19.
A patient with recurrent epistaxis and excessive bleeding after dental extraction is suspected of having von Willebrand disease. Which investigation most directly measures the amount of von Willebrand factor in plasma?
20.
Which pair represents the major thrombotic microangiopathies?
21.
Which laboratory pattern is most characteristic of hemophilia A?
22.
A patient has a prolonged aPTT that corrects after mixing the patient's plasma with normal plasma. What does this finding most strongly suggest?
23.
A patient with severe sepsis develops bleeding from venepuncture sites and signs of organ dysfunction. Laboratory testing shows thrombocytopenia, prolonged PT and aPTT, and increased D-dimer. What is the most likely diagnosis?
24.
A student compares the coagulation pathways using laboratory tests. A disorder selectively impairing tissue factor–factor VII activity would be detected most directly by which test?
25.
A patient with von Willebrand disease has a reduction in factor VIII activity despite having no primary defect in the factor VIII gene. Which function of von Willebrand factor explains this finding?
26.
A patient receiving an antifibrinolytic drug for recurrent mucosal bleeding develops a complication related to excessive preservation of fibrin clots. Which adverse effect is of greatest concern?
27.
A patient with disseminated intravascular coagulation develops acute renal and neurological dysfunction. Histological examination of small vessels is most likely to show which lesion?
28.
Two brothers have recurrent deep muscle bleeding after minor trauma. One has deficient factor VIII activity and the other has deficient factor IX activity. Which feature is expected in both disorders?
29.
Which function of von Willebrand factor is most important in primary hemostasis?
30.
A patient has simultaneous abnormalities affecting both the intrinsic and extrinsic coagulation pathways. Which activated factor represents the major point at which these pathways converge before thrombin generation?
31.
A patient with thrombotic thrombocytopenic purpura develops platelet-rich microvascular thrombi. Deficiency of which protein is most closely associated with this condition?
32.
A 24-year-old woman reports recurrent gum bleeding and prolonged bleeding after dental extraction. Her clinician suspects von Willebrand disease and wants to determine whether the abnormality is quantitative or functional. Which investigation is most useful in addition to measurement of von Willebrand factor quantity?
33.
A patient receiving hematopoietic supportive therapy for thrombocytopenia develops peripheral edema and worsening fluid retention. The drug acts by promoting megakaryocyte development. Which agent is most likely responsible?
34.
Desmopressin can reduce bleeding in selected patients with mild hemophilia A primarily by increasing the release of:
35.
A patient with newly diagnosed immune thrombocytopenia develops clinically significant mucosal bleeding and requires a relatively rapid increase in platelet count. Which treatment is most appropriate for achieving this effect?
36.
Which description best corresponds to type 1 von Willebrand disease?
37.
A 19-year-old woman has recurrent epistaxis and excessive menstrual bleeding. Platelet count is normal. Testing shows that von Willebrand factor is present but functions abnormally. Which classification best fits this disorder?
38.
A patient with suspected disseminated intravascular coagulation has thrombocytopenia and prolonged PT and aPTT. Which additional result would further support consumption of coagulation substrate?
39.
A patient with advanced malignancy develops diffuse bleeding, thrombocytopenia and prolonged coagulation tests. Peripheral smear shows fragmented erythrocytes. What is the best explanation for the red-cell abnormality?
40.
Tranexamic acid reduces bleeding mainly through which mechanism?
