Picture
Hypo and Hyper
Factors
100

This German scientist is often called the father of modern pathology.

Rudolf Wirchow

100

Which platelet receptor defect is present in patients with Bernard-Soulier syndrome?

GpIb

100

How are PT and aPTT altered in hemolytic uremic syndrome?

No change

200

What syndrome does Dorian suffer from?


Dense granulocyte disease

200

A 23-year-old woman is 25 weeks pregnant but has not felt fetal movement for the past 3 days. Three weeks later, she developed dyspnea and cyanosis. Physical examination revealed a temperature of 37°C, a pulse of 106 beats/min, respiratory rate of 23 beats/min, and a blood pressure of 80/40 mm Hg. The skin of the entire body was covered with extensive bruising. Laboratory examination revealed prolonged PT and aPTT, thrombocytopenia, significantly decreased plasma fibrinogen, and fibrin degradation products. No bacteremia was found. What is the most likely cause of the bleeding?
A. Platelet aggregation defect
B. High vascular fragility
C. Decreased platelet production
D. Dissemeniated intravascular coagulation

DIC

200

A 16-year-old girl reports frequent nosebleeds and easy bleeding gums since childhood. She also complains of menorrhagia. Physical examination is unremarkable. Laboratory tests: hemoglobin 14.1 g/dL; hematocrit 42.5%; MCV 90 μm3; platelets 277,400/mm3; and leukocytes 5920/mm3. Her platelets do not aggregate in response to ACE and adrenaline. She has glycoprotein IIb/IIIa deficiency.
What disease does this girl suffer from?

Glanzmann's thrombasthenia

300

What process of hemostasis is reflected in this painting by Marc Chagall, and what components of that process do the artist, his lover, and the green meadows play?

Adhesion
Meadow - collagen
Painter - von Willebrand factor
Lover - platelet

300

A 21-year-old woman with hereditary protein C deficiency developed pulmonary embolism and was prescribed anticoagulant therapy. Two weeks after starting this therapy, her mental status changed dramatically, and she developed difficulty speaking and swallowing. Cerebral angiography revealed occlusion of the left middle cerebral artery.  Laboratory findings were hemoglobin 13 g/dL, platelets 65,400/mm3, white blood cells 5924/mm3, PT 12 seconds, and aPTT 51 seconds. The decision was made to discontinue the prescribed therapy.  Which of the following anticoagulants is most likely to have caused these symptoms?

A. Aspirin  

B. Heparin
C. Urokinase
E. Warfarin

Heparin

300

A 15-year-old girl presents to her endocrinologist complaining of easy bruising and bleeding. She has had menometrorrhagia since her menarche at age 13. Physical examination reveals joint deformity and decreased range of motion in the ankles, knees, and wrists. Laboratory findings include hemoglobin 11.8 g/dL, hematocrit 35.1%, platelet count 267,000/mm3, white blood cell count 5960/mm3, normal PT13 and prolonged aPTT, and normal platelet function tests. A 1:1 dilution of the patient’s plasma with plasma from a healthy donor corrected the aPTT. Which of the following is the most likely diagnosis?


A Antiphospholipid syndrome
B Hemophilia B
C Thrombotic thrombocytopenic purpura 

D von Willebrand disease

Hemophilia B

400

According to unproven rumors, this is the city that inspired Winston Churchill and the Wachowski brothers, and it is in this city that the incidence of venous thrombosis is highest.

Leiden

400

A 38-year-old woman presented to the hospital complaining of bloody blisters on her tongue and petechiae on her extremities. According to the patient, she has not had any recent viral infections and is not currently taking any medications, but she has been drinking tonic water daily due to recent painful leg cramps. Laboratory tests show thrombocytopenia, normal leukocyte and erythrocyte counts, normal aPTT and PT. A peripheral blood smear shows a significant decrease in platelets without schistocytes.

Which of the following is the most likely cause of thrombocytopenia?


A. Anti-GPIIb/IIIa antibody-mediated platelet destruction
B. Kinin-dependent antibody-mediated platelet destruction
C. Parvovirus bone marrow suppression
E. Hemolytic uremic syndrome

Antibody-mediated kinin-dependent platelet destruction


400

A clinical study was conducted in adult patients diagnosed with microangiopathic hemolytic anemia. A subgroup of patients who had fever or diarrhea before the initial diagnosis of anemia was excluded. Schistocytes and von Willebrand factor multimer accumulation were found in the blood smear of these patients.
Which protein deficiency is most likely present in these patients?

ADAMTS13

500

The patient presented to the local polyclinic complaining of fever, acute, painful polyarthritis, abdominal pain, and bloody vomiting. Urinalysis revealed microscopic hematuria and elevated serum IgA levels. What pathology is present in this patient?

Sholeyn-Henoch purpura

500

On a farm in rural Scotland, a 4-year-old dairy cow developed spontaneous bleeding from the gums and nostrils and died despite the efforts of a veterinarian. Similar symptoms and death were recorded in about a dozen other cows in the following days. Laboratory tests performed while they were alive showed prolonged PT and aPTT with normal platelet counts. Necropsy showed extensive internal bleeding. According to the farmers, the herd had recently been fed on clover hay in an open-air barn.

What is the most likely cause of the bleeding?
1. Inhibition of platelet aggregation by toxic alkaloids
2. Inhibition of vitamin K epoxide reductase
3. Abnormal collagen synthesis
4. Overproduction of plasmin and increased fibrinolysis

 

Vitamin K epoxide reductase inhibition

500

Desmopressin, a synthetic analogue of vasopressin, is widely used in the treatment of von Willebrand disease. It is believed that by acting on endothelial V2 receptors, it leads to the production of von Willebrand factor and, by this mechanism, alleviates the course of the disease.  In which of the diseases of the hemostasis system would its use be indicated?

Hemophilia A