A 44-year-old man in acute distress is noted to have multiple ecchymoses of the skin that have developed over the past 48 hours. His prothrombin time (PT) is 38 seconds and partial thromboplastin time (PTT) 55 seconds. A CBC shows a WBC count of 5300/microliter, hemoglobin 8.1 g/dL, hematocrit 24.9%, MCV 99 fL, and platelet count 16,300/microliter. His D-Dimer test is very high. Which of the following is the most likely diagnosis?
A Hemophilia A
B Afibrinogenemia
C Vitamin K deficiency
D vonWillebrand disease
E Disseminated intravascular coagulopathy
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A 31-year-old woman has developed easy bruisability, even with minor trauma, to her skin over the past month. On physical examination she has multiple contusions of varying ages seen over her torso and extremities. She has no difficulty with movement. No neurologic deficits are noted. Her PT and PTT are normal. Her Hgb is 13.3 g/dL with Hct 42.1% and MCV 95 fL, platelet count 25,000/microliter, and WBC count is 7500/microliter. Which of the following is the most likely diagnosis?
B Idiopathic thrombocytopenic purpura
C vonWillebrand disease
D Acute promyelocytic leukemia
E Anti-phospholipid syndrome
A clinical study is performed with subjects who have thrombocytopenia <10,000/microliter. These patients are observed to have defects in coagulation with spontaneous bleeding characterized by multiple petechiae and purpura and stool samples positive for occult blood. Which of the following functions is most likely to remain largely intact in these patients?
A Adherence to injured vascular walls
B Conversion of fibrinogen to fibrin
C Induction of vasoconstriction
D Increase in surface sites for coagulation
E Release of substances that neutralize heparin
A clinical study is conducted with subjects who have thrombocytopenia <15,000/microliter. It is observed that hemostasis is impaired in these subjects. Which of the following laboratory test abnormalities is most likely to be seen in these subjects?
A Prolonged prothrombin time
B Positive anticardiolipin antibody
C Prolonged partial thromboplastin time (PTT)
D Prolonged bleeding time
E Elevated D-dimer
F Increased vonWillebrand factor multimers
A 35-year-old woman has noted small hemorrhages on her skin increasing in number over the past month. On physical examination she has petechiae involving skin surfaces of her torso and extremities. Laboratory studies show a normal PT and normal PTT. Her Hgb is 9.8 g/dL, Hct 29.4%, MCV 80 fL, platelet count 276,300/microliter, and WBC count 7290/microliter. Platelet function studies are normal. Which of the following is the most likely diagnosis?
A vonWillebrand disease
B Uremia
C Scurvy
D Aspirin ingestion
E Chronic alcoholism
A 12-year-old boy has had worsening problems with joint mobility involving his arms and legs, particularly his knees and ankles, for the past 6 years. He has been receiving therapy for this condition. On physical examination he has no visible petechiae or areas of purpura. Laboratory studies show that his prothrombin time is 12 seconds and his partial thromboplastin time is 52 seconds. After addition of an equivalent aliquot of normal plasma, his PTT is 40 seconds. His Hgb is 12.9 g/dL, platelet count 238,500/microliter, and WBC count 6620/microliter. Which of the following is the most appropriate statement regarding his clinical condition?
A There is no family history for this condition
B His sisters are affected as well
C He has had episodes of mucocutaneous bleeding
D Transfusion of factor VIII concentrate is helpful
E He has an underlying liver disease
A 54-year-old woman has a pre-operative workup performed prior to a total abdominal hysterectomy for uterine leiomyomata. On physical examination she has no abnormal findings. Laboratory studies show a Hgb of 12.1 g/dL, Hct 35.8%, MCV 90 fL, platelet count of 1,000,000/microliter, and WBC count 3850/microliter. Her prothrombin time is 13 seconds and partial thromboplastin time 28 seconds. Which of the following is the most likely diagnosis?
A Anti-phospholipid syndrome
B vonWillebrand disease
C Hereditary spherocytosis
D Chronic liver disease
E Myeloproliferative disorder
A 22-year-old woman has had multiple episodes of sudden dyspnea, sometimes following in a day or two by chest pain, over the past 6 years. Following these episodes pulmonary ventilation/perfusion scans have shown perfusion defects. She now has developed lower extremity pain over the past day. On physical examination there is absence of the posterior tibial pulse on the right. A thrombectomy is performed. A deficiency of which of the following blood proteins is most likely to be the cause for her findings?
A Alpha-2-macroglobulin
B Fibrinogen
C alpha-2-antiplasmin
D Antithrombin III
E Factor V
A 69-year-old woman has had flank pain and a high fever for a week. On physical examination her temperature is 38 C. There is left costovertebral angle tenderness. A urine culture is positive for Esherichia coli. She then has a drop in blood pressure and in urine output. A blood culture grows Escherichia coli. Her prothrombin time is 34 seconds and her partial thromboplastin time 59 seconds. Her platelet count is 11,000/microliter. Her D-dimer is elevated. Which of the following is the most likely mechanism for development of her laboratory findings?
A Venous thrombosis
B Endothelial cell injury
C Increased factor VIII levels
D Splenic sequestration
E Arterial bleeding
A clinical study is conducted comparing patients diagnosed with vonWillebrand disease (VWD) to patients diagnosed with hemophilia A. Both groups of patients have documented problems of hemostasis over a 10 year period. Which of the following complications is most likely to be seen more frequently in the patients with hemophilia A?
A Petechiae
B Epistaxis (nosebleed)
C Excessive bleeding from wounds
D Hemarthrosis (bleeding into joints)
E Menorrhagia (excessive menstrual hemorrhage)
A 35-year-old man undergoes a pre-operative workup for a hernia repair. He has no history of a bleeding disorder. On physical examination there are no abnormal findings other than the right inguinal hernia. Laboratory studies show Hgb 14.8 g/dL, Hct 45%, MCV 92 fL, WBC count 8500/microliter, and platelet count 275,000/microliter. His prothrombin time is 12.6 seconds, and his partial thromboplastin time is 48 seconds. After a mixing study with addition of normal plasma to his plasma in a 1:1 ratio, the PTT is 25 seconds. A deficiency of which of the following coagulation factors is most likely to be present in this man?
A IX
B VIII
C XII
D X
E XIII
A clinical study is conducted to determine which laboratory test will best aid in distinguishing the coagulopathy of severe liver disease with that of disseminated intravascular coagulopathy (DIC). The records of two groups of patients, one group with DIC and one with liver diease, are reviewed. Which of the following laboratory tests is most likely to be abnormal with disseminated intravascular coagulopathy (DIC)?
A Prothrombin time
B Platelet count
C Partial thromboplastin time
D D-dimer test
E Factor XIII assay
A 35-year-old man has had fever for the past 5 days. Yesterday he developed difficulty speaking. On physical examination he has petechial hemorrhages on his lower extremities. His temperature is 37.7 C. Laboratory studies show Hgb 8.4 g/dL, Hct 25.9%, MCV 100 fL, platelet count 9000/microliter, and WBC count 9800/microliter. Examination of his peripheral blood smear shows schistocytes. His prothrombin time is 18 seconds and his partial thromboplastin time is 44 seconds. His serum creatinine is 2.6 mg/dL, haptoglobin is 2 mg/dL, and total bilirubin 5.5 mg/dL. He died despite plasmapheresis. At autopsy, platelet thrombi are observed in the small arteries of the kidneys, heart, and brain. Which of the following is the most likely diagnosis?
A Thrombotic thrombocytopenic purpura (TTP)
B vonWillebrand disease (vWD)
C Malaria
D Systemic lupus erythematosus (SLE)
E Alcoholic liver disease
F Antiphospholipid syndrome
A clinical study is conducted with subjects from 15 to 50 years of age who have bleeding disorders. The medical records of these patients are reviewed to determine what complications they developed. A subset of these subjects is found to have the complication of hemarthroses. Which of the following laboratory test findings is most likely to be present in this subset?
A Factor V deficiency
B Prothrombin time twice normal control
C Positive protamine sulfate test
D Platelet count of 100,000
E Factor VIII deficiency
A 28-year-old woman has noted the appearance of pinpoint hemorrhages on her skin for the past 3 weeks. On physical examination she has petechiae on her torso and extremities. She is afebrile. Laboratory studies show Hgb 12.8 g/dL, Hct 38.4%, MCV 88 fL, platelet count 17,250/microliter, and WBC count 5285/microliter. No schistocytes are seen on her peripheral blood smear. Her prothrombin time is 12.2 seconds and her partial thromboplastin time is 26 seconds. Which of the following is most likely to cause her disease?
A Splenomegaly
B Megakaryocytic hypoplasia
C Chronic renal failure
D Anti-platelet antibodies
E Blood loss
A clinical study is performed with subjects who are found to have laboratory test abnormalities involving the coagulation system. Review of patient records reveals that some of these subjects have had problems with bleeding while others have not. Patients with which of the following laboratory test abnormalities are most likely to have bleeding?
A Lupus anticoagulant
B Factor XII deficiency
C Factor IX deficiency
D Prekallikrein deficiency
E High-molecular-weight kininogen deficiency
A 30-year-old man experiences excessive bleeding following a wisdom tooth extraction. There is a family history of similar problems. His mother had a history of menorrhagia. His sister has frequent nosebleeds. Laboratory studies on this man show he has a normal PT, PTT, and platelet count. Which of the following is the most likely diagnosis?
A Antiphospholipid syndrome
D Hemophilia B
E Protein C deficiency
A 46-year-old man has noted increasing numbers of blotchy areas of purple discoloration on his skin over the past 5 months. On physical examination there are areas of purpura but no petechiae. Laboratory studies show Hgb 12.5 g/dL, Hct 37.4%, MCV 85 fL, platelet count 251,300/microliter, and WBC count 8140/microliter. His prothrombin time is 22 seconds and his partial thromboplastin time is 25 seconds. Which of the following is most likely to explain his findings?
A Heparin therapy
B Lupus anticoagulant
C Factor XI deficiency
D Severe vonWillebrand disease
E Factor VII deficiency
A 38-year-old man has frequent nosebleeds that are difficult to stop. On physical examination there are no abnormal findings. Laboratory studies show Hgb 13.5 g/dL, Hct 40.4%, MCV 84 fL, platelet count 248,500/microliter, and WBC count 7350/microliter. His prothrombin time is 12.1 seconds and partial thromboplastin time is 26 seconds. Which of the following conditions is he most likely to have?
A Massive hepatic steatosis
B Coumadin therapy
E Hemophilia B
A 22-year-old woman has the sudden onset of dyspnea. On physical examination she has right leg swelling and tenderness. Laboratory studies show her prothrombin time is 12.4 seconds with partial thromboplastin time 53 seconds. Her plasma is mixed in a ratio of 1:1 with normal plasma and the partial thromboplastin time does not normalize. Which of the following is the most likely diagnosis?
B Disseminated intravascular coagulopathy
C Antithrombin III deficiency
D Vitamin K deficiency
E Hemophilia A
A 35-year-old woman undergoes an elective vaginal hysterectomy. During this procedure, there is significant loss of blood with oozing from small vessels. Coagulation tests not ordered would have shown a normal PT, PTT, and platelet count--but a prolonged bleeding time. What over-the-counter drug the patient took just prior to the procedure is the most probable cause for these findings?
A Acetaminophen
B Phenylpropanolamine
C Codeine
D Ephedrine
E Acetylsalicylic acid
A 53-year-old man experiences a severe nosebleed. On physical examination he has a ruddy complexion. He is afebrile. His stool is positive for occult blood. Laboratory studies show Hgb 19.7 g/dL, Hct 59.8%, MCV 93 fL, platelet count 445,000/microliter, and WBC count 22,460/microliter. His prothrombin time is 13 seconds and his partial thromboplastin time is 27 seconds. Which of the following abnormalities of the coagulation system is most likely to cause his bleeding?
A Anti-platelet antibodies
B Bernard-Soulier syndrome
C Abnormal platelet function
D Metastatic carcinoma
In an experiment, initiation of the coagulation system is studied by the addition of various compounds to aliquots of normal patient plasma. After addition of a compound, the prothrombin time is measured with one aliquot and the partial thromboplastin time with another aliquot. Addition of which of the following compounds is most likely to produce clotting, as measured by the prothrombin time?
A Fibrinogen
B Tissue factor
C Factor VIII
D Factor VII
E Factor XII
F Factor XIII
A 33-year-old woman is in the intensive care unit following delivery of a stillborn fetus. Fetal demise had occurred 3 weeks previously. On physical examination her vital signs include T 37.2 C, P 101/minute, RR 18/minute, and BP 80/44 mm Hg. Laboratory studies show Hgb 10.5 g/dL, Hct 31.7%, MCV 96 fL, platelet count 45,000/microliter, and WBC count 11,700/microliter. Examination of her peripheral blood smear shows that schistocytes are present. Which of the following laboratory tests is most likely to confirm her diagnosis?
A Hemoglobin electrophoresis
B Serum ferritin
C D-dimer
D Bone marrow biopsy
E Reticulocyte count
A 52-year-old man has an episode of severe hematemesis. On physical examination there are no abnormal findings. An abdominal CT scan shows a small, nodular liver. Laboratory studies show Hgb 10.5 g/dL, Hct 31.1%, MCV 70 fL, platelet count 199,400/microliter, and WBC count 7370/microliter. His prothrombin time is 24 seconds and his partial thromboplastin time is 33 seconds. Which of the following is most likely to explain his findings?
A Myeloproliferative disorder
C Abnormal fibrinolysis
D Factor VIII deficiency
A 19-year-old man develops a sudden episode of mental confusion and weakness. On physical examination he has 4/5 motor strength in his right arm. Laboratory studies show Hgb 14.5 g/dL, Hct 44.4%, MCV 89 fL, platelet count 227,800/microliter, and WBC count 6480/microliter. His prothrombin time is 12 seconds and partial thromboplastin time 25 seconds. A year later he develops deep venous thrombosis of his left lower leg. A mutation involving a gene encoding for which of the following is most likely to be present in this man?
A Prothrombin
B Factor VIII
C Factor IX
D vonWillebrand factor
E Factor XIII
A 15-year-old boy has the sudden onset of dyspnea. On physical examination he has vital signs with T 37.1 C, P 90/minute, RR 25/minute, and BP 100/60 mm Hg. A pulmonary ventilation/perfusion scan shows a large right perfusion defect. Laboratory studies show Hgb 13.9 g/dL, Hct 42%, MCV 91 fL, platelet count 238,200/microliter, and WBC count 6170/microliter. His prothrombin time is 12.7 seconds and partial thromboplastin time 26 seconds. Which of the following conditions is he most likely to have?
A High molecular weight kininogen deficiency
B Alpha-1-antitrypsin deficiency
C Hemophilia A
D Factor V Leiden mutation
E vonWillebrand disease
A 54-year-old woman who has been hospitalized for two weeks develops swelling of her right leg. Raising the leg elicits pain. The leg feels warm on palpation. An ultrasound examination reveals evidence for thrombosis in the deep veins of the right leg. Within a day, she has the sudden onset of dyspnea, and a day later has left-sided chest pain on inspiration. Which of the following is the best initial therapy for this woman?
A Coumadin
B Heparin
C Aspirin
D Prednisone
E Urokinase
A clinical study is conducted with subjects who are adult women from 30 to 45 years of age who have been taking oral contraceptives for at least 10 years. The medical records for these subjects are reviewed to determine if vascular problems occurred. It is observed that there is a slight but significant increase in thrombotic and thromboembolic events in these subjects, compared to an age-matched control group not taking oral contraceptives. Which of the following mechanisms is most likely to increase the risk for thrombosis in this group of women?
A Anti-platelet antibody
B Anti-cardiolipin antibody
C Elevated factor VIII level
D Homocysteinemia
E Antithrombin III deficiency
A 45-year-old woman undergoes mitral valve replacement with a mechanical prosthesis as treatment for chronic rheumatic valvulitis with mitral stenosis. She is started on coumadin therapy. Which of the following statements regarding the use of this medication is most appropriate?
A Coumadin acts by inhibiting factor XII
B If she also takes aspirin, the dosage of coumadin must be decreased
C Renal disease will affect the response to coumadin
D Her anticoagulation should be monitored with the partial thromboplastin time (PTT)
E The action of coumadin is immediate after ingestion
A 7-year-old boy has the sudden onset of dyspnea. On physical examination he has T 37 C, P 95/minute, RR 27/minute, and BP 110/65 mm Hg. A head & neck CT scan shows a large soft tissue hematoma in his left lateral neck which impinges upon the pharynx and larynx. Later in life he develops hemarthroses with joint ankylosis. Which of the following laboratory profiles is most characteristic for his underlying disease?
A Increased PT, normal PTT, low Factor VIIIAHF, decreased vWFAg
B Increased PT, increased PTT, low Factor VIIIAHF, normal vWFAg
C Normal PT, increased PTT, low Factor VIIIAHF, normal vWFAg
D Normal PT, increased PTT, low Factor IX, normal vWFAg
E Normal PT, increased PTT, low Factor VIIIAHF, decreased vWFAg
A 60-year-old woman develops pain in her left leg over the past week. On physical examination she has swelling of her left leg. A doppler ultrasound scan shows decreased flow with deep venous thrombosis. A pulmonary ventilation/perfusion scan is normal. Which of the following sequential therapies is most appropriate for treatment of this woman?
A Aspirin, then coumadin
B Intravenous heparin, then coumadin
C A fibrinolytic agent, then coumadin
D Subcutaneous heparin, then coumadin
E A fibrinolytic agent, then aspirin
A clinical study is performed with subjects from ages 10 to 90 who developed deep venous thrombosis to determine risk factors for the thrombotic event. Demographic, clinical, and laboratory findings are reviewed and statistically analyzed for significance. These subjects are compared to an age-matched control group of patients without a history of deep venous thrombosis. Which of the following underlying conditions is most likely to increase the risk for deep venous thrombosis?
A Chronic renal failure
B Pulmonary emphysema
C Diverticulosis
D Adenocarcinoma
E Ischemic heart disease
A 22-year-old G2 P1 woman gives birth following an uncomplicated pregnancy to a term male infant. There is prolonged bleeding upon separation of the umbilical cord. There is prolonged bleeding with circumcision. Laboratory studies show Hgb 12.9 g/dL, Hct 39%, MCV 95 fL, platelet count 188,400/microliter, and WBC count 8155/microliter. The prothrombin time is 29 seconds, partial thromboplastin time 50 seconds, and the thrombin time is elevated. Which of the following is the most likely diagnosis?
C Sticky platelet syndrome
E Afibrinogenemia
A 69-year-old man is admitted to the hospital for a coronary artery bypass grafting procedure. On physical examination there are no abnormal findings. Pre-operative laboratory studies show Hgb 13.8 g/dL, Hct 41.3%, MCV 86 fL, platelet count 222,200/microliter, and WBC count 5695/microliter. His prothrombin time is 14 seconds and his partial thromboplastin time is 56 seconds. He has no history of a bleeding or thrombotic disorder. He is most likely to have a deficiency of which of the following coagulation factors?
A VII
C IX
D XII
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