Practice questions coagulopathy

Practice questions

Coagulopathy

© Jun Wang, MD, PhD

1. Use this case for the next four questions. A 3-year-old boy presents with oozing bleeding from his mouth after a fall 2 hours ago. He has a history of easy bruising since birth. He had an episode of low-grade fever 1 week ago, that was relieved after his parents gave him aspirin. His past history is otherwise unremarkable. Vaccination is up to dates. Physical examination reveals a few bruises on his legs. No other abnormality is seen. Laboratory tests reveal an aPTT of 36 second (normal range: 24.4-32.3 second). His CBC, PT are all normal. Mixing study for aPTT is 30.5 second (normal range: 28.2-38.1 second). What additional study would be most helpful for making a diagnosis?

A. Liver function tests

B. Renal function tests

C. Serum autoantibodies for coagulation factors

D. Serum vitamin B12 and folate levels

E. von Wellibrand factor activity, factors VIII and IX

2. A 3-year-old boy presents with oozing bleeding from his mouth after a fall 2 hours ago. He has a history of easy bruising since birth. He had an episode of low-grade fever 1 week ago, that was relieved after his parents gave him aspirin. His past history is otherwise unremarkable. Vaccination is up to dates. Physical examination reveals a few bruises on his legs. No other abnormality is seen. Laboratory tests reveal an aPTT of 36 second (normal range: 24.4-32.3 second). His CBC, PT are all normal. Mixing study for aPTT is 30.5 second (normal range: 28.2-38.1 second). Abnormity of what factor is most likely causing the findings about aPTT?

A. Factor VII deficiency

B. Factor VIII deficiency

C. Factor IX deficiency

D. Factor VIII autoantibody

E. Platelet GPIIb/IIIa antibody

3. A 3-year-old boy presents with oozing bleeding from his mouth after a fall 2 hours ago. He has a history of easy bruising since birth. He had an episode of bloody diarrhea 1 week ago, but has recovered without medical treatment. His past history is otherwise unremarkable. Vaccination is up to dates. Physical examination reveals a few bruises on his legs. No other abnormality is seen. Laboratory tests reveal an aPTT of 36 second (normal range: 24.4-32.3 second). His CBC, PT are all normal. Mixing study for aPTT is 30.5 second (normal range: 28.2-38.1 second).

Additional test results include: Factor VIII at 0.25 IU/ml (normal 0.6-1.5 IU/ml), Factor IX at 0.92 IU/ml (normal 0.6-1.5 IU/ml), von Willebrand factor antigen at 21 IU/dL (normal 50-200 IU/dL). What is most likely the diagnosis?

A. Hemolytic uremic syndrome

B. Hemophilia A, acquired

C. Hemophilia A, congenital

D. Hemophilia B

E. Von Willebrand disease

4. A 3-year-old boy presents with oozing bleeding from his mouth after a fall 2 hours ago. He has a history of easy bruising since birth. He had an episode of low-grade fever 1 week ago, that was relieved after his parents gave him aspirin. His past history is otherwise unremarkable. Vaccination is up to dates. Physical examination reveals a few bruises on his legs. No other abnormality is seen. Laboratory tests reveal an aPTT of 36 second (normal range: 24.4-32.3 second). His CBC, PT are all normal. Mixing study for aPTT is 30.5 second (normal range: 28.2-38.1 second). Abnormality of which hemostasis activity is most likely associated with his hemorrhagic episode?

A. Common coagulative pathway

B. Fibrinolysis

C. Extrinsic coagulative pathway

D. Platelet adhesion to vascular collagen

E. Vascular fragility

5. Use this case for the next four questions. A 5-year-old presents with painful rapid left knee swelling and loss of function for 1 hour, after a fall in the playground. He has a history of easy bruising since birth. He has a few male members in his maternal side with easy bruising. Physical examination reveals edema of left lower leg and joint effusion. The left knee is held flexed. Lachman’s test, valgus, varus stress tests are all negative. Anterior drawer test is inconclusive. No other significant abnormality is seen. His CBCs are within normal range. No morphological abnormality is noted. His PT is 11 seconds (normal 8.8-12.7 second), aPTT is 40 second (normal 23-29 second), aPTT after mixing with same quantity of pooled plasma is 25 second. What additional abnormality is most likely found?

A. Factor V

B. Factor VII

C. Factor VIII

D. Factor X

E. Von Willibrand factor

6. A 5-year-old presents with painful rapid left knee swelling and loss of function for 1 hour, after a fall in the playground. He has a history of easy bruising since birth. He has a few male members in his maternal side with easy bruising. Physical examination reveals edema of left lower leg and joint effusion. The left knee is held flexed. Lachman’s test, valgus, varus stress tests are all negative. Anterior drawer test is inconclusive. No other significant abnormality is seen. His CBCs are within normal range. No morphological abnormality is noted. His PT is 11 seconds (normal 8.8-12.7 second), aPTT is 40 second (normal 23-29 second), aPTT after mixing with same quantity of pooled plasma is 25 second. What is most likely causing his knee swelling?

A. Acute bacterial infection

B. Anterior cross ligament injury

C. Intra-articular bleeding

D. Medial collateral ligament injury

E. Synovial inflammation

7. A 5-year-old presents with painful rapid left knee swelling and loss of function for 1 hour, after a fall in the playground. He has a history of easy bruising since birth. He has a few male members in his maternal side with easy bruising. Physical examination reveals edema of left lower leg and joint effusion. The left knee is held flexed. Lachman’s test, valgus, varus stress tests are all negative. Anterior drawer test is inconclusive. No other significant abnormality is seen. His CBCs are within normal range. No morphological abnormality is noted. His PT is 11 seconds (normal 8.8-12.7 second), aPTT is 40 second (normal 23-29 second), aPTT after mixing with same quantity of pooled plasma is 25 second. Additional tests reveal normal level factor IX, but reduced factor VIII. What is most likely causing his elongated aPTT?

A. Bacterial toxin activity

B. Congenital factor VIII deficiency

C. Factor VIII inhibitor

D. Platelet antibody

E. Ultra large von Willebrand multimer

8. A 5-year-old presents with painful rapid left knee swelling and loss of function for 1 hour, after a fall in the playground. He has a history of easy bruising since birth. He has a few male members in his maternal side with easy bruising. Physical examination reveals edema of left lower leg and joint effusion. The left knee is held flexed. Lachman’s test, valgus, varus stress tests are all negative. Anterior drawer test is inconclusive. No other significant abnormality is seen. His CBCs are within normal range. No morphological abnormality is noted. His PT is 11 seconds (normal 8.8-12.7 second), aPTT is 40 second (normal 23-29 second), aPTT after mixing with same quantity of pooled plasma is 25 second. What is most likely the diagnosis?

A. Acute bacterial arthritis

B. Anterior cross ligament tear

C. Hemophilic hemarthrosis

D. Juvenile idiopathic arthritis

E. Medial collateral ligament tear

9. Use this case for the next three questions. A 40-year-old woman presents with severe menorrhagia for 4 months. She has a history of Graves disease, and is now treated with thionamides. She has no history of easy bleeding or bruising. Her family history is unremarkable. Her vital signs are within normal ranges. Physical examination reveals generalized pallor. No other abnormality is noted. Laboratory tests results include: hemoglobin, 5.1 g/ dL (12.0-15.5 g/dL); mean corpuscular volume (MCV), 86.7 fL (81.6-98.3 fL); white cell count, 7.2 × 10⁹/L (3.5-10.5 × 10⁹/L); platelet count, 315 × 10⁹/L (150-450 × 10⁹/L); prothrombin time 11.5 s (normal 11-13.5 s); activated partial thromboplastin time 39.0 s (normal 25-35 s), and fibrinogen 174 mg/dL (150-400 mg/dL). APTT after mixing with same quantity of pooled plasma is 36 second. Additional tests reveal normal level von Willebrand factors. What is most likely causing her menorrhagia?

A. Factor VII deficiency

B. Factor VIII deficiency

C. Uterine neoplasms

D. Vitamin K deficiency

E. von Willebrand factor abnormality

10. A 40-year-old woman presents with severe menorrhagia for 4 months. She has a history of Graves disease, and is now treated with thionamides. She has no history of easy bleeding or bruising. Her family history is unremarkable. Her vital signs are within normal ranges. Physical examination reveals generalized pallor. No other abnormality is noted. Laboratory tests results include: hemoglobin, 5.1 g/ dL (12.0-15.5 g/dL); mean corpuscular volume (MCV), 86.7 fL (81.6-98.3 fL); white cell count, 7.2 × 10⁹/L (3.5-10.5 × 10⁹/L); platelet count, 315 × 10⁹/L (150-450 × 10⁹/L); prothrombin time 11.5 s (normal 11-13.5 s); activated partial thromboplastin time 38.0 s (normal 25-35 s), and fibrinogen 174 mg/dL (150-400 mg/dL). APTT after mixing with same quantity of pooled plasma is 36 second. Additional tests reveal normal level von Willebrand factors.

Additional test reveal a factor VIII level at 0.2 U/ml (normal 0.5-1.5 U/ml). What is most likely causing her reduced level of factor VIII?

A. Antibodies against factor VIII

B. Factor VIII gene mutation

C. Over consumption of factor VIII

D. Vitamin K deficiency

E. von Willebrand factor abnormality

11. A 40-year-old woman presents with severe menorrhagia for 4 months. She has a history of Graves disease, and is now treated with thionamides. She has no history of easy bleeding or bruising. Her family history is unremarkable. Her vital signs are within normal ranges. Physical examination reveals generalized pallor. No other abnormality is noted. Laboratory tests results include: hemoglobin, 5.1 g/ dL (12.0-15.5 g/dL); mean corpuscular volume (MCV), 86.7 fL (81.6-98.3 fL); white cell count, 7.2 × 10⁹/L (3.5-10.5 × 10⁹/L); platelet count, 315 × 10⁹/L (150-450 × 10⁹/L); prothrombin time 11.5 s (normal 11-13.5 s); activated partial thromboplastin time 38.0 s (normal 25-35 s), and fibrinogen 174 mg/dL (150-400 mg/dL). APTT after mixing with same quantity of pooled plasma is 36 second.

Additional test reveal a factor VIII level at 0.2 U/ml (normal 0.5-1.5 U/ml). What is most likely the diagnosis?

A. Acquired hemophilia

B. Hemophilia B

C. Liver disease associated coagulopathy

D. Vitamin K deficiency

E. von Willebrand factor abnormality

12. A 2-year-old boy presents with excessive nose bleeding. He has a history of easy bruising. A few of his uncles of the maternal side have similar presentation. Physical examinations reveals general pallor with a few hematomatous changes on his arms and legs. Blood is seen in his nose. Laboratory test results include: hemoglobin at 8.2 g/dL (normal 10.5–12.7 g.dL), PT 11.2 second (normal 9.2-13 second), aPTT 82.5 second (normal 23.5-33.5 second). His white cells and platelets are within normal range. No morphological abnormality is seen. Additional tests reveals a factor VIII level at 0.9 U/ml (normal 0.5-1.5 U/ml), factor IX level at 0.1 IU/ml (normal 0.6-1.5 IU/ml), von Willebrand factor antigen at 105 IU/dL (normal 50-200 IU/dL). Screening test for inhibitors is negative. What is most likely the diagnosis?

A. Hemophilia A

B. Hemophilia B

C. Immune thrombocytopenic purpura

D. Lupus coagulopathy

E. Vitamin K deficiency

13. Use this case for the next two questions. A 16-year-old girl presents with rapidly developed painful right knee swollen for 2 hours. She does not remember any specific trauma. She has a history of easy bruising and amenorrhea. A few of his uncles of the maternal side have some kind of bleeding disorder that she and her mother are not quite clear. Family history of her paternal side is unremarkable. Physical examinations reveals short statue, underdeveloped breast, general pallor with a few bruises on her arms and legs. Her right knee is markedly swollen with extension limitation. The knee is held at a flexed position. Special exams for the knee joint injuries including Lachman’s test are all negative. Laboratory test results include: hemoglobin at 7.6 g/dL (normal 10.7–13.5g.dL), PT 10.5 second (normal 9.2-13 second), aPTT 78 second (normal 23.5-33.5 second). Her white cells and platelets are within normal range. No morphological abnormality is seen. Additional tests reveals a factor VIII level at 0.15 U/ml (normal 0.5-1.5 U/ml), factor IX level at 0.9 IU/ml (normal 0.6-1.5 IU/ml), von Willebrand factor antigen at 98 IU/dL (normal 50-200 IU/dL). Screening test for inhibitors is negative. What is most likely the diagnosis?

A. Acquired coagulopathy

B. Hemophilia A

C. Hemophilia B

D. Vitamin K deficiency

E. Von Willebrand disease

14. A 16-year-old girl presents with rapidly developed painful right knee swollen for 2 hours. She does not remember any specific trauma. She has a history of easy bruising and amenorrhea. A few of his uncles of the maternal side have some kind of bleeding disorder that she and her mother are not quite clear. Family history of her paternal side is unremarkable. Physical examinations reveals short statue, underdeveloped breast, general pallor with a few bruises on her arms and legs. Her right knee is markedly swollen with extension limitation. The knee is held at a flexed position. Special exams for the knee joint injuries including Lachman’s test are all negative. Laboratory test results include: hemoglobin at 7.6 g/dL (normal 10.7–13.5g.dL), PT 10.5 second (normal 9.2-13 second), aPTT 78 second (normal 23.5-33.5 second). Her white cells, platelets, transferrin and total iron binding capacity are within normal range. No morphological abnormality is seen. Additional tests reveals a factor VIII level at 0.15 U/ml (normal 0.5-1.5 U/ml), factor IX level at 0.9 IU/ml (normal 0.6-1.5 IU/ml), von Willebrand factor antigen at 98 IU/dL (normal 50-200 IU/dL). Screening test for inhibitors is negative. What additional abnormality she most likely has?

A. Autoimmune disorder, such as systemic lupus erythematosus

B. Chronic liver disease

C. Iron deficiency

D. Turner syndrome

E. Warfarin toxicity

15. Use this case for the next two questions. A 56-year-old farmer presents with hematuria for a few days. He denies pain with voiding. He had a history of spreading rodenticides in his farm for a few weeks. He has a history of hypertension. He denies any medication usage. His family history is unremarkable. He has been drinking beers one can a day for 35 years. Physical examination is unremarkable. Urinalysis reveals 75 red blood cells per high-power field, but no white cells, casts or renal tubular cells are seen. His CBC is within normal range. He has a PT of 45.5 second (normal range 11.9–14.5 seconds), an activated partial thromboplastin time (PTT) = 68.7 seconds (normal 24.6–34.6 seconds), and an international normalized ratio (INR) of 7.8. Mixing studies are negative for inhibitors. Additional studies reveals deficiency of factors II, VII, and IX. No other abnormality is seen. What is most likely causing his hematuria?

A. Alcoholic liver disease

B. Exaggerated hypertension

C. Neoplasm of urinary tract

D. Urinary tract infection

E. Vitamin K deficiency

16. A 56-year-old farmer presents with hematuria for a few days. He denies pain with voiding. He has a history of hypertension. For the recent few weeks, he was helping his friends handling rodenticides in their farms. He denies any medication usage. His family history is unremarkable. He has been drinking beers one can a day for 35 years. Physical examination is unremarkable. Urinalysis reveals 75 red blood cells per high-power field, but no white cells, casts or renal tubular cells are seen. His CBC is within normal range. He has a PT of 45.5 second (normal range 11.9–14.5 seconds), an activated partial thromboplastin time (PTT) = 68.7 seconds (normal 24.6–34.6 seconds), and an international normalized ratio (INR) of 7.8. Mixing studies are negative for inhibitors. Additional studies reveals deficiency of factors II, VII, IX and X. No other abnormality is seen.

Additional tests reveal serum warfarin is positive. What is most likely causing deficiencies of his coagulation factors?

A. Autoantibodies against multiple coagulation factors

B. Inhibition of vitamin K reductase

C. Inhibition of vitamin-K-dependent carboxylase

D. Malabsorption of fat

E. Over consumption of coagulation factors

17. Use this case for the next two questions. A 62-year-old man presents with sudden onset of dyspnea and right chest pain. He had a history of prostate cancer 5 years ago and received prostatectomy and radiation therapy. His past medical history and family history is unremarkable. He has been drinking 2 glasses of whisky each day for 40 years. He smokes cigarette one and a half pack a day for 35 years. Physical examination reveals pale conjunctiva and icteric sclerae. Breathing sound is reduce at the right chest. No other abnormality is noted during physical examination. Radiologic examinations reveals large amount of right pleural fluid accumulation. Diagnostic thoracentesis reveals bloody effusion. His laboratory test results include: Hemoglobin at hemoglobin at 8.4 g/dL (normal 12–18g/dL), PT 44 second (normal 9.2-13 second), INR 4.4, aPTT 49 second (normal 23.5-33.5 second), total bilirubin, 3.2 mg/dL (normal 0-1 mg/dL), direct bilirubin 1.7 mg/dL (normal 0-0.4 mg/dL);  alanine aminotransferase, 1250 U/L (normal 10-55 U/L); aspartate aminotransferase, 420 U/L (normal 10-40 U/L). Mixing studies are negative for inhibitors. PSA is within normal range. His white cells, platelets, FDP and fibrinogen are within normal range. No morphological abnormality is seen. What is most likely associated with his clinical presentations?

A. Acute bronchopneumonitis

B. Coagulative factor deficiencies

C. Metastatic prostate cancer to lung

D. Primary malignant neoplasm of lung

E. Trauma

18. A 62-year-old man presents with sudden onset of dyspnea and right chest pain. He had a history of prostate cancer 5 years ago and received prostatectomy and radiation therapy. His past medical history and family history is unremarkable. He has been drinking 2 glasses of whisky each day for 40 years. He smokes cigarette one and a half pack a day for 35 years. Physical examination reveals pale conjunctiva and icteric sclerae. Breathing sound is reduce at the right chest. No other abnormality is noted during physical examination. Radiologic examinations reveals large amount of right pleural fluid accumulation. Diagnostic thoracentesis reveals bloody effusion. His laboratory test results include: Hemoglobin at hemoglobin at 8.4 g/dL (normal 12–18g/dL), PT 44 second (normal 9.2-13 second), INR 4.4, aPTT 49 second (normal 23.5-33.5 second), total bilirubin, 3.2 mg/dL (normal 0-1 mg/dL), direct bilirubin 1.7 mg/dL (normal 0-0.4 mg/dL);  alanine aminotransferase, 1250 U/L (normal 10-55 U/L); aspartate aminotransferase, 420 U/L (normal 10-40 U/L). Mixing studies are negative for inhibitors. PSA is within normal range. His white cells, platelets, FDP and fibrinogen are within normal range. No morphological abnormality is seen. What additional abnormality is most likely found?

A. Inhibitors of coagulation factors

B. Low level of factor VII

C. Low level of factor VIII

D. Low level of factor XIII

E. Ultra large von Willebrand multimers

19. Use this case for the next two questions. A 53-year-old male farmer presents with sudden onset of left hypochondrium and flank pain. He has no significant past medical history. He did mention he was distributing rodenticides a few days ago in his farm. Physical examination reveals no significant findings. Radiological examination reveals enlarge spleen, as well as signs of splenic infarction. His laboratory test results include: platelet 97 x 10⁹/L (normal 150-450 x 10⁹/L), PT 18 second (normal 9.2-13 second), aPTT 37 second (normal 23.5-33.5 second), fibrinogen 34.2 mg/dL (normal 150-400 mg/dL), fibrinogen degradation products 750 microgram/ml (normal < 10 microgram/ml), and LDH 635 U/L (normal <270 U/L). His hemoglobin and white cell counts are within normal range. Peripheral blood smears reveals increased number of immature nucleated cells with intracellular needle like crystals. Red cells and platelets are morphologically unremarkable. Molecular studies reveal t(15;17). What is causing his splenic infarction?

A. Activation of platelets due to ultra large von Willebrand multimers

B. Activation of platelets due to endothelial injury

C. Activation of platelets by autoantibodies

D. Warfarin toxicity induced coagulopathy

E. Widespread activation of coagulation cascades

20. A 53-year-old male farmer presents with sudden onset of left hypochondrium and flank pain. He has no significant past medical history. He did mention he was distributing rodenticides a few days ago in his farm. Physical examination reveals no significant findings. Radiological examination reveals enlarge spleen, as well as signs of splenic infarction. His laboratory test results include: platelet 97 x 10⁹/L (normal 150-450 x 10⁹/L), PT 18 second (normal 9.2-13 second), aPTT 37 second (normal 23.5-33.5 second), fibrinogen 34.2 mg/dL (normal 150-400 mg/dL), fibrinogen degradation products 750 microgram/ml (normal < 10 microgram/ml), and LDH 635 U/L (normal <270 U/L). His hemoglobin and white cell counts are within normal range. Peripheral blood smears reveals increased number of immature nucleated cells with intracellular needle like crystals. Red cells and platelets are morphologically unremarkable. Molecular studies reveal t(15;17). What is most likely the diagnosis associated with his presentations?

A. Acute promyelocytic leukemia

B. Acquired hemophilia

C. Thrombotic thrombocytopenic purpura

D. Vitamin K deficiency

E. Von Willibrand disease

Answers
Back to bleeding disorders
Back to contents