Exam 34: Disorders of Secondary Hemostasis

George Jones is admitted to the hospital with abdominal pain. Upon physical examination, the attending physician notices a yellow ring around his green eyes. George has a significant swelling in his abdomen and has been suffering from swollen joints for the last month. The physician runs a battery of labs. The results follow: Based on this information, from what is George most likely suffering?

A 5-year-old boy has his blood drawn for platelet aggregation studies. Ristocetin aggregation (RIPA) comes back abnormal with all other agonists displaying normal aggregation. VWF antigen is within normal limits. What is the most likely reason for this discrepancy?

Which of the following clinical symptoms correlates with a severe form of hemophilia A or B?

Deficiencies of the fibrin-forming proteins often have a delayed bleeding symptom that results from which of the following?

Deficiency of coagulation proteins is defined as a decrease in:

Which of the following laboratory assays would be useful in identifying the presence of primary fibrinolysis?

A known hemophiliac has developed an inhibitor. The physician in charge of his care wants to know how much inhibitor is present to properly treat him. What two lab tests would be ordered to assess this?

What should be the next course of action in investigating these results?

Screening tests for the evaluation of VWD include the platelet count, APTT, PT, bleeding time, and/or PFA-100. Which of the following results would be evident in most cases of VWD?

Hemophilia A is caused by a deficiency in which of the following proteins?

Which congenital deficiency(ies) is(are) suspected with a normal platelet count, an abnormal PT and APTT, and normal PFA-100 and thrombin time?

F-VIII deficiency and F-IX deficiency are classified as what category of disorder?

A physician has confirmed von Willebrand's disease in a patient. Further testing is needed to determine which subtype the patient has. Multimer analysis is performed and comes back normal. What is the most probable explanation for this?

What is the most probable diagnosis?

What is the inheritance pattern of hemophilia A?

Explain how liver disease can be differentiated from DIC through laboratory test analysis.

What type of VWD is the only type to have a decreased platelet count?

Interpret the following results: PT: 11.8 sec (RR: 11.2-13.8 sec) INR: 1.07 APTT: 57 sec (RR: 23-32 sec) Closure time: increased Ristocetin aggregation: decreased aggregation Based on this information, what is the most likely disorder?

How does von Willebrand's disease (VWD) differ from Bernard-Soulier syndrome? Correlate the pathophysiology of both disorders in your response. Name at least two laboratory tests that differentiate each.

Explain the inheritance pattern of the following disorders and how it contributes to the clinical manifestations of each: a. Hemophilia A b. Hemophilia B c. von Willebrand's disease d. Bernard-Soulier syndrome