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Disease states: fibrinolytic system and DIC

10 ASCP MLS practice questions on Disease states: fibrinolytic system and DIC, part of Hematology. The first 10 are below; the first 5 with answers and explanations (tap Show answer after you have chosen), the rest in Learn with a free account.

  1. Question 1 · Hard

    A 4-month-old girl has a firm, purple mass on her shoulder and chest wall that has grown quickly over 2 weeks and is now tense and bruised. She has no fever, and blood cultures are negative. Results: hemoglobin 9.0 g/dL (90 g/L), platelets 12 × 10^3/µL (12 × 10^9/L), fibrinogen 68 mg/dL (0.68 g/L; reference 200-400), PT 15.9 s (reference 11.0-13.5 s), APTT 37 s (reference 25-35 s), D-dimer 9.2 µg/mL FEU (9.2 mg/L FEU; reference below 0.5). The film shows a few schistocytes. Urea and creatinine are normal. What best explains these results?

    1. ASepsis-induced disseminated intravascular coagulation
    2. BImmune thrombocytopenia of infancy
    3. CLocalized consumption in a vascular tumor
    4. DTypical hemolytic uremic syndrome
    Show answer

    Answer: C · Localized consumption in a vascular tumor

    Severe thrombocytopenia, low fibrinogen and a high D-dimer show consumption, and the rapidly growing vascular mass is where it happens: this is the Kasabach-Merritt phenomenon. Platelets are trapped and activated on the abnormal endothelium of a kaposiform hemangioendothelioma or tufted angioma, setting off local clotting and fibrinolysis; common infantile hemangiomas do not do this. Sepsis is unlikely without fever and with negative cultures. ITP and typical HUS do not lower fibrinogen or raise D-dimer this much, and HUS would damage the kidneys. The consumption settles only when the tumor responds to treatment, and platelet transfusions can make the lesion swell.

    • A: There is no fever, cultures are negative, and the clotting is centered on the enlarging mass.
    • B: ITP lowers platelets only; fibrinogen and D-dimer stay normal.
    • D: HUS damages the kidneys, and urea and creatinine are normal; fibrinogen is usually not consumed.
  2. Question 2 · Medium

    A patient has schistocytes and a platelet count of 22 × 10^3/µL (22 × 10^9/L). Which coagulation pattern favors TTP over DIC?

    1. ALong thrombin time and raised FDP
    2. BProlonged PT and APTT, low fibrinogen
    3. CNormal PT, APTT and fibrinogen
    4. DLow fibrinogen and a raised D-dimer
    Show answer

    Answer: C · Normal PT, APTT and fibrinogen

    TTP is caused by severe ADAMTS13 deficiency, which leaves ultra-large VWF multimers that build platelet-rich thrombi. Clotting factors are not consumed, so the PT, APTT and fibrinogen are usually normal. DIC consumes factors and fibrinogen, so it prolongs clotting times and raises D-dimer sharply. An ADAMTS13 activity below 10% supports TTP and calls for urgent plasma exchange.

    • A: A long thrombin time with raised FDP reflects fibrinogen loss and lysis, as in DIC.
    • B: Prolonged clotting times with low fibrinogen point to consumption, as in DIC.
    • D: Low fibrinogen with a high D-dimer is the classic DIC picture.
  3. Question 3 · Hard

    A septic patient has platelets 72 × 10^3/µL (72 × 10^9/L), a strongly raised D-dimer, a PT 4 s above the upper reference limit and fibrinogen 130 mg/dL (1.3 g/L). ISTH overt DIC points: platelets 50-100 = 1, < 50 = 2; D-dimer moderate rise = 2, strong rise = 3; PT 3-6 s long = 1, > 6 s = 2; fibrinogen < 100 mg/dL = 1. What is the score?

    1. A6, compatible with overt DIC
    2. B7, compatible with overt DIC
    3. C5, compatible with overt DIC
    4. D4, below the overt DIC cutoff
    Show answer

    Answer: C · 5, compatible with overt DIC

    Platelets of 72 score 1, a strong D-dimer rise scores 3, a PT 4 s long scores 1, and fibrinogen of 130 mg/dL scores 0 because it is above 100 mg/dL (1.0 g/L). The total is 1 + 3 + 1 + 0 = 5. A score of 5 or more is compatible with overt DIC; a lower score is repeated in 1 to 2 days. The score is used only in patients with a disorder known to cause DIC.

    • A: A score of 6 wrongly gives a point for a fibrinogen above 100 mg/dL.
    • B: A score of 7 also scores the platelets as if they were below 50.
    • D: A score of 4 counts the D-dimer as a moderate rise, not a strong one.
  4. Question 4 · Easy

    Which fragment is released only when plasmin digests fibrin that factor XIIIa has cross-linked?

    1. AFragment X
    2. BD-dimer
    3. CFibrinopeptide A
    4. DFragment E
    Show answer

    Answer: B · D-dimer

    Factor XIIIa joins the D domains of neighbouring fibrin monomers with covalent bonds. When plasmin digests this cross-linked fibrin, it releases D-dimer and larger fragments that contain it. Plasmin acting on fibrinogen or on fibrin that is not cross-linked gives fragments X, Y, D and E, but no D-dimer. A raised D-dimer therefore shows that thrombin made fibrin and plasmin then broke it down, as happens in DIC.

    • A: Fragment X is an early plasmin product of fibrinogen as well as of fibrin.
    • C: Fibrinopeptide A is cut from fibrinogen by thrombin, before any fibrinolysis.
    • D: Fragment E is released from fibrinogen as well as from fibrin.
  5. Question 5 · Hard

    A 66-year-old man with septic shock receives no heparin. From day 1 to day 2: platelets 240 to 92 × 10^3/µL (240 to 92 × 10^9/L); fibrinogen 520 to 265 mg/dL (5.2 to 2.65 g/L; reference 200-400); PT 13.0 to 16.8 s (reference 11.0-13.5); D-dimer 1.2 to 7.5 µg/mL FEU (reference < 0.5). What is the best interpretation?

    1. AEvolving DIC with fibrinogen consumption
    2. BDilution of factors by intravenous fluids
    3. CDIC excluded, as fibrinogen stays normal
    4. DVitamin K deficiency from antibiotics
    Show answer

    Answer: A · Evolving DIC with fibrinogen consumption

    Fibrinogen is an acute-phase protein, so in sepsis it often starts high, and a fall of about half into the reference range still shows heavy consumption. The falling platelet count, lengthening PT and sharply rising D-dimer complete the picture of developing DIC. Trends are more useful than single values, which is why DIC scores are repeated daily. Treatment is aimed at the underlying sepsis.

    • B: Dilution does not explain a sharply rising D-dimer.
    • C: A fibrinogen that has halved shows consumption even inside the reference range.
    • D: Vitamin K deficiency prolongs the PT but does not lower platelets or raise D-dimer.
  6. Question 6 · Easy

    In acute DIC, the platelet count and fibrinogen fall mainly because they are:

    1. ADiluted by intravenous fluids
    2. BCleared by antiplatelet antibodies
    3. CUsed up in widespread microthrombi
    4. DUnderproduced by marrow and liver

    Answer and explanation in LearnFree account

  7. Question 7 · Medium

    A 64-year-old man with metastatic pancreatic cancer has had two venous thromboses in a month. PT and APTT are normal, fibrinogen is 430 mg/dL (4.3 g/L; reference 200-400), platelets are 165 × 10^3/µL (165 × 10^9/L) and D-dimer is 4.8 µg/mL FEU (4.8 mg/L FEU; reference < 0.5). These results best fit:

    1. AHeparin-induced thrombocytopenia
    2. BChronic compensated DIC
    3. CAcute decompensated DIC
    4. DPrimary hyperfibrinolysis

    Answer and explanation in LearnFree account

  8. Question 8 · Hard

    At 34 weeks, a woman with new hypertension has platelets 64 × 10^3/µL (64 × 10^9/L), AST 210 U/L (reference 10-40), LD 980 U/L (reference 120-250), schistocytes and a normal glucose. PT, APTT and fibrinogen are normal, and D-dimer is mildly raised. These results best fit:

    1. AAcute fatty liver of pregnancy
    2. BAcute obstetric DIC
    3. CHELLP syndrome
    4. DImmune thrombocytopenia

    Answer and explanation in LearnFree account

  9. Question 9 · Medium

    In acute DIC, the thrombin time is prolonged mainly because of:

    1. AConsumption of factor VII by tissue factor
    2. BLow fibrinogen and interfering FDP
    3. CLoss of factors V and VIII
    4. DToo few platelets to support clotting

    Answer and explanation in LearnFree account

  10. Question 10 · Medium

    Which finding best separates primary hyperfibrinolysis from DIC?

    1. AProlonged PT and APTT
    2. BRaised fibrin(ogen) degradation products
    3. CNormal platelets and near-normal D-dimer
    4. DLow fibrinogen and a long thrombin time

    Answer and explanation in LearnFree account

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