Thrombotic Disorders



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2015

Thrombotic Disorders

Janna M. Journeycake, MD
1. You are consulting on a new patient who was recently diagnosed with deep vein thrombosis (DVT) of the right femoral vein. He is a 15-year-old Caucasian male who weighs 90 kg and who developed leg pain and swelling after a football injury to his knee that required bracing. After the DVT was diagnosed, he was placed on heparin infusion and transitioned to warfarin. Current INR is 2.4. Thrombophilia testing was performed prior to discharge from the hospital.

Factor V Leiden

Homozygous normal

Prothrombin gene mutation

Homozygous normal

Factor VIII

200%

Protein S activity

40%

Protein C activity

30%

Antithrombin

85%

Lupus anticoagulant

Negative

Based on these results, the most likely contributed to his thrombosis is

A. Inherited protein C deficiency

B. Inherited protein S deficiency

C. Immobility

D. Obesity

E. Elevated FVIII


2. A 16-year-old previously healthy African-American girl presents to the emergency room with a 3-day history of leg pain and progressive swelling such that she can no longer walk. Her entire left leg is edematous and discolored compared with the right leg. It is warm to touch. She denies any trauma, but she is on the basketball team at school. Her only medication includes oral contraceptive pills (OCPs), which she started at age 12 for menorrhagia. She weighs 90 kg. Doppler sonogram shows complete occlusion of the left iliac, femoral, superficial femoral, and popliteal veins. She is treated with anticoagulation with good clinical results. Follow-up magnetic resonance imaging to assess extent of the thrombus will likely show

A. Occlusion up to the left renal vein

B. Right iliac artery overriding the left iliac vein

C. Congenital inferior vena cava (IVC) disruption

D. Ovarian mass compressing the left iliac vein

E. No intra-abdominal problems


3. A 5-month-old Caucasian female had open heart surgery 7 days ago. She is on an unfractionated heparin infusion at prophylactic dosing. Her total white blood cell (WBC) count was 13,000/mL with 50% segmented neutrophils, 3% bands, 10% monocytes, and 37 % lymphocytes. Hemoglobin was 10.5 g/dl and platelet count 45,000/mL. Her prothrombin time (PT) is 13 seconds, partial thromboplastin time (PTT) is 45 seconds, fibrinogen is 350 mg/dl, and D-dimers are mildly elevated. Her ALT is 35 U/L, AST 37 U/L, and creatinine 1.0 mg/dl. She has a catheter in her right femoral vein that is not flushing adequately and a Doppler sonogram shows partial occlusion of the right femoral vein. The next best step in this patient’s management is to

A. Test for heparin-induced thrombocytopenia without altering current anticoagulation.

B. Remove the femoral catheter.

C. Discontinue unfractionated heparin and begin warfarin.

D. Test for heparin-induced thrombocytopenia and begin an alternative anticoagulant agent.

E. Discontinue unfractionated heparin and begin enoxaparin.


4. The best alternative anticoagulant medication to use in the baby described in question 3 is

A. Lepirudin

B. Argatroban

C. Rivaroxaban

D. Clopidogrel

E. No alternative anticoagulation is necessary


5. You are managing a teenage girl on chronic warfarin therapy. The INR has been stable for the past 6 months but is now 1.4. She denies missing any doses except for the day before her routine lab test, and a pill count suggests that no other doses have been missed. Which is the most likely reason for the drop in INR?

A. She has started to drink alcohol on the weekends.

B. She was prescribed an antibiotic for a sinus infection the week before.

C. She has a gene polymorphism making her resistant to warfarin.

D. She started a diet this month consisting of mostly fruits and vegetables.

E. She missed the dose the day before the test.


6. Which of the following statements comparing LMWH and unfractionated heparin (UFH) provides an explanation of the best initial anticoagulant therapy to choose in a neonate with a catheter-related thrombosis. The laboratory results are as follows: creatinine 0.9, platelets 450, antithrombin activity 50%. A head ultrasound is negative for bleeding, and the child has no need for any invasive procedures.

A. LMWH should be chosen because it does not rely on the need for antithrombin.

B. LMWH should be chosen because it does not need to be monitored.

C. UFH should be chosen because it can be safely prescribed in a patient with renal insufficiency.

D. UFH should be chosen because it has shorter half-life.

E. LMWH should be chosen because there is no risk for HIT.

7. Which aspect of the endothelium has anticoagulant properties?

A. Release of thrombomodulin

B. Release of von Willebrand factor (VWF) and cleavage by ADAMTS-12

C. Vasoconstriction

D. Release of tissue factor
8. A term infant born to a diabetic mother presents with hematuria and a flank mass. A renal ultrasound demonstrates bilateral renal vein thrombosis and extension into the IVC. His creatinine is 0.4, but his urine output is lower than expected. The team consults you to ask about the use of systemic thrombolysis. What would be the strongest contraindication for thrombolysis?

A. Platelet count of <50, 000

B. Guaiac positive stool

C. Decreased creatinine clearance

D. Surgery for placement of central venous catheter in past 3 days

E. Plasminogen deficiency

9. A 4-year-old white female is brought to your emergency room at a large tertiary care medical center for nose bleeds, easy bruising, and altered mental status. She has been staying with her grandparents while her parents are out of town. She has never had any bleeding issues in the past and even underwent tonsillectomy 6 months ago without incident. Family history is negative for bleeding disorders, but her grandfather is on warfarin for atrial fibrillation. Laboratory testing is pending and a head CT is being performed. You suspect warfarin toxicity due to accidental ingestion of the grandfather’s medications. The CT confirms intracranial hemorrhage. The complete blood count (CBC) shows hemoglobin of 8.9 g/dl and platelets of 250, 000. PT is markedly prolonged and corresponds to INR of 10.5. PTT is also prolonged at 60 seconds. What is the best management for this child?

A. Give oral vitamin K at a dose of 5 mg.

B. Give fresh frozen plasma (FFP) at a dose of 10 cc/kg.

C. Give Recombinant activated factor VIIa (rFVIIa) at a dose of 90 mcg/kg.

D. Give a prothrombin complex concentrate (PCC) at a dose of 50 units/kg.

E. Gastric lavage


10. A 15-year-old girl is transferred from an outside emergency room with the diagnosis of pneumonia for which her primary care provider prescribed Augmentin 3 days prior. She has low-grade fever and is hypoxic and requiring 35% FiO2 per facemask. She complains of chest pain over her left ribs. Outside chest X ray shows an infiltrate in the left lower lobe. Her heart rate is 140, respiratory rate is 28, blood pressure is 110/60, and temperature is 38.2 °C. In addition to the Augmentin, she is taking a combined OCP that was prescribed 2 months ago due to dysmenorrhea. Family history is positive for her father developing a DVT at age 44 when he traveled overseas. No thrombophilia testing had even been done in family. What is the best next step in determining the diagnosis in this child?
A. D-dimer testing

B. CT angiogram of chest

C. Echocardiogram

D. Activated protein C resistance (APCR) testing

E. Protein S activity testing
11. Which inherited thrombophilia is most likely to lead to a venous thromboembolic event prior to age 40?

A. Heterozygous factor V Leiden gene mutation

B. Double heterozygous MTHFR polymorphism

C. Homozygous prothrombin gene mutation

D. Heterozygous antithrombin deficiency

E. Homozygous PAI-1 mutation


12. Which of the following patients has the strongest risk for developing a DVT?

A. 3-year-old Asian male recovering from intussusception surgery

B. 10-year-old white male in maintenance for acute lymphoblastic leukemia therapy whose central venous catheter was removed 3 months ago due to infection

C. A premature 1-month-old white female on parenteral nutrition

D. 16-year-old white male in the hospital for laparoscopic cholecsytectomy

E. 5-year-old black female in the hospital for asthma exacerbation whose father had DVT at age 40


13. A 15-year-old boy with life-threatening pulmonary embolism is being treated with the thrombolytic agent tPA. Which of the following complications indicates that thrombolysis should be discontinued?

A. Blood oozing from the line site

B. Fibrinogen < 100 mg/dl

C. Platelets < 50,000/mm3

D. Decline in Hgb > 2 g/dl

E. Elevation of D-dimers


14. A 32-year-old Caucasian male comes to your office with his 4-year-old daughter for counseling. He was recently diagnosed with a DVT involving the lower extremity and was found to be heterozygous for the factor V (FV) Leiden gene mutation. The most appropriate counseling to provide for the family is to

A. Advocate for testing for FV Leiden in the child immediately.

B. Educate the family about signs and symptoms of thromboembolic disease.

C. Begin the child on prophylactic aspirin.

D. Consider screening the child for every known cause of thrombosis.

E. Counsel the family that the child is not at risk for thrombosis during childhood.

15. A 12-year-old white male was diagnosed with a DVT of the right lower extremity after knee surgery. A thrombophilia evaluation was performed due to the relatively low risk expected for a DVT in this patient. He has been on anticoagulation for past 3 months and you are asked to provide a recommendation for duration of therapy. Which of the following scenarios would require prolonged or indefinite anticoagulation therapy?

A. Lupus anticoagulant was positive at diagnosis and remains positive.

B. At diagnosis the FVIII activity level was 300% and it is now 150%.

C. He is heterozygous for the FV Leiden mutation.

D. His protein C activity is 60%.

E. His mother had a DVT in the postpartum period.




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