Weeks 1-4 Review

Iron homeostasis/Iron deficiency anemias

100

An 8-month-old female is brought to the pediatrician’s office because her parents are worried about her decrease in activity. She produces 8 wet diapers daily and is exclusively breastfed. On physical examination, the child appears lethargic. Complete blood count shows anemia.

Which of the following additional findings would most likely be present in this patient?

A) Decreased mean corpuscular volume

B) Decreased total iron-binding capacity

C) Hypocellular marrow

D) Increased ferritin level

E) Increased reticulocyte count

F) Macrocytosis

A) Decreased mean corpuscular volume

An infant who is exclusively breastfed and presents with decreased activity and anemia most likely has iron-deficiency anemia.

Iron-deficiency anemia typically results in a decreased mean corpuscular volume, decreased serum ferritin level, and increased total iron-binding capacity.

200

An 8-month-old boy is brought to the clinic by his mother due to lethargy, poor feeding, and abdominal swelling that have worsened over the past 2 months. The pregnancy and birth were unremarkable. Physical examination reveals poor weight gain, hepatosplenomegaly, and jaundice. Complete blood cell count results show a hemoglobin (Hb) of 4.2 g/dL and a reticulocyte count of 10%. Hb electrophoresis shows marked elevation of fetal Hb (HbF), increased Hb alpha 2 (HbA2), and absence of adult Hb (HbA).

Which of the following is the most likely diagnosis?

A) α-Thalassemia minor

B) β-Thalassemia major

C) β-Thalassemia minor

D) Glucose-6-phosphate dehydrogenase deficiency

E) Hb Barts disease

F) HbH disease

B) β-Thalassemia major

β-Thalassemia major presents with severe hemolytic anemia, jaundice, hepatosplenomegaly, and poor growth.

β-Thalassemia major is caused by a genetic defect in the β-chain gene on both copies of chromosome 11, resulting in an absence or marked decrease of β-chain production.

300

A previously healthy 38-year-old woman is brought to the emergency department by her husband because of left-sided weakness for the past 4 hours. As she was getting dressed this morning, she was suddenly unable to button up her shirt. When she showed her husband, he noticed that she was confused. She has a 3-year history of diabetes mellitus, for which she takes metformin. She had a knee operation 1 month ago. Her temperature is 38.9°C (102°F), pulse is 98/min, respirations are 17/min, and blood pressure is 138/85 mm Hg. She is confused and oriented only to person. Neurologic examination shows diminished muscle strength on the left side. There are scattered petechiae over the chest, arms, and legs. Laboratory studies show:A peripheral smear shows numerous schistocytes. Further evaluation is most likely to show which of the following findings?

A. RBC agglutination on direct Coombs test

B. Mutations of alternative complement pathway genes on genetic testing

C. Decreased ADAMTS13 activity in serum

D. Antibodies against platelet membrane glycoproteins on serologic testing

E. Elevated fibrin degradation products in serum

C

400

A previously healthy 44-year-old man comes to his physician because of frequent urination and increased thirst for several weeks. Physical examination shows darkened skin and a firm mass in the right upper quadrant. His blood glucose is 220 mg/dL. A photomicrograph of a specimen obtained on liver biopsy is shown. Which of the following best describes the pathogenesis of the disease process in this patient?

A. Upregulation of erythropoietin production

B. Absence of β-globin synthesis

C. Absence of a serine protease inhibitor

D. Defective transferrin receptor binding

E. Defective serum copper transportation

D

500

A 63-year-old woman comes to the physician for evaluation of worsening fatigue and an unintentional 6.8-kg (15-lb) weight loss over the past 2 months. She also reports having had an unusual appetite for soil and clay for several months. She has a history of osteoarthritis of the knees, for which she takes acetaminophen. Her pulse is 116/min and blood pressure is 125/84 mm Hg. Physical examination shows diffuse teeth abrasions and dirt in the sublingual folds. Further evaluation of this patient is most likely to show which of the following findings?

A. Positive stool guaiac test

B. Elevated serum thyroid-stimulating hormone concentration

C. Elevated serum lead concentration

E. Decreased CD4+ T-lymphocyte count

Positive Schilling test

A

Hemolysis/Hemolytic anemias

100

An 84-year-old man is admitted to the hospital for a 2-day history of bloody diarrhea. He is hemodynamically stable, but testing reveals a hemoglobin of 8.0 g/dL. An RBC transfusion is ordered. Soon after the transfusion begins, the patient complains of nausea and flank and chest pain. Vitals show a temperature of 103°F (39.4°C), pulse 125/min, respirations are 20/min, and blood pressure 95/65 mm Hg. After stopping the transfusion, it is noted that the RBC unit that was being administered is blood type AB negative, and the patient’s blood type is A positive.

Which type of hypersensitivity reaction is responsible for this patient’s posttransfusion signs and symptoms?

1. Type I

2. Type II

3. Type III

4. Type IV

Type II

1. ABO-incompatible red cell transfusions cause acute hemolytic transfusion reactions, which present with fever, flank pain, and circulatory collapse.

2. The underlying mechanism of acute hemolytic transfusion is a type II hypersensitivity reaction in which IgG antibodies bind to donor RBCs, leading to complement-mediated hemolysis.

200

A 14-year-old male is brought to the clinic because of yellow discoloration of his eyes for 1 day. This developed 4 days after he had a 2-day illness marked by fever and watery diarrhea. Laboratory studies show the following:

Hemoglobin:  11 g/dL

Hematocrit:  32%

Platelet count:  250,000/mm3

Leukocyte count:  7000/mm3

Reticulocyte count:  4.8%

Indirect bilirubin:  3.6 mg/dL (↑)

A Coombs test is negative. 

A peripheral blood smear with crystal violet staining is shown.

Which of the following is the most likely cause of this patient's anemia?

1. Autoimmune hemolytic anemia

2. Glucose-6-phosphate dehydrogenase deficiency

3. Hereditary spherocytosis

4. Sickle cell disease

5. Sideroblastic anemia

Glucose-6-phosphate dehydrogenase deficiency

1. G6PD deficiency is an X-linked deficiency of a central enzyme in the hexose monophosphate shunt pathway. G6PD deficiency makes RBCs sensitive to oxidative stress and subsequently causes a hemolytic anemia with exposure to oxidative stresses.

2. Blood smear showing Heinz bodies are indicative of G6PD as well

300

A 14-year-old male is brought to a pediatrician by his mother because the whites of his eyes appear yellow. The patient has no complaints and no significant medical history. The exam is normal except for icteric sclerae. Laboratory tests show a total bilirubin level of 4.5 mg/dL and a direct bilirubin level of 4.0 mg/dL, with normal transaminases and alkaline phosphatase.

Which of the following is the most likely diagnosis?

1. Auto-immune hepatitis

2. Crigler-Najjar syndrome

3. Dubin-Johnson Syndrome

4. Gilbert syndrome

Dubin-Johnson Syndrome

1. Dubin-Johnson syndrome is an autosomal-recessive disorder caused by an impairment of the hepatocyte transport system for the removal of conjugated anions into bile.

2. Often presents with direct hyperbilirubinemia in a child/teenager without transaminase elevations and normal alkaline phosphatase

400

A 38-year-old male presents to the emergency department because of a 3-week history of recurrent episodes of fevers, night sweats, intense headaches, and weakness. He recently spent a month in southeast Asia and did not take any prophylactic medications before or during his trip. He says that the fevers happen every 24 to 48 hours, but he has not been able to identify a clear pattern. Physical examination reveals hepatosplenomegaly. During the examination, he has a generalized seizure. T2-weighted fluid-attenuated inversion recovery MRI of the brain is shown.

Which of the following parasites is most likely causing this patient’s symptoms?

1. Babesia microti

2. Plasmodium falciparum

3. Plasmodium malariae

4. Plasmodium ovale

5. Plasmodium vivax

Plasmodium falciparum

1. Cerebral malaria presents with irregularly cyclic fevers, anemia, and hepatosplenomegaly, as well as cerebral involvement.

2. P falciparum is the only cause of malaria that presents with irregularly cyclic fevers and has cerebral involvement.

500

A 22-year-old woman presents to her family physician because of increasing fatigue and pale appearance. She reports that her urine appears brown each morning. There is no history of major medical illness. Laboratory studies show RBC of 3 million/mm3 and hemoglobin 10 g/dL.

Which of the following best describes the most likely mechanism of erythrocyte injury in this condition?

1. A defective cytoskeleton-membrane tethering protein

2. Complement-mediated hemolysis

3. Increased oxidative injury by H2O2 due to decreased NADPH

4. Phagocytosis of RBCs due to complement fixation

5. Point mutation in the hemoglobin β-chain

Complement-mediated hemolysis

1. PNH is an acquired clonal stem cell disorder characterized by intravascular hemolysis with anemia and “cola-colored” urine in the morning.

2. The mild respiratory acidosis that occurs during sleep activates complement and lyses RBCs because glycosylphosphatidylinositol (GPI) is defective and cannot bind CD55 and CD59 and protect the RBCs from complement-mediated lysis.

Coagulation/Bleeding disorders

100

A 55-year-old man presents with large reddish bruises. He underwent mitral valve replacement surgery several months prior and was subsequently started on an anticoagulant that requires frequent monitoring. He was advised to reduce his grapefruit intake due to interactions with the medication, but he has not done so. Physical examination reveals multiple ecchymoses on his arms and trunk.

The medication most likely prescribed for this patient affects which of the following coagulation factors?

1. Factor V

2. Factor I

3. Factor VII

4. Factor XIII

5. Factor XI

Factor VII

1. A portion of warfarin is metabolized by CYP450 3A4; therefore, excessive consumption of grapefruit (an inhibitor of CYP450 3A4) may increase the risk of bleeding in patients taking warfarin.

2. Warfarin acts by preventing the normal synthesis and γ-carboxylation of the vitamin K–dependent clotting factors II, VII, IX, and X and proteins C and S via vitamin K antagonism.

200

A 23-year-old woman presents to the clinic because of calf pain. She has had two episodes of deep venous thromboses in 5 years, one of which led to pulmonary embolus. She also had three miscarriages. Laboratory testing shows low platelets, a positive rapid plasma reagin test result, and a negative fluorescent treponemal antibody test result.

Which of the following additional laboratory findings is most likely in this patient?

1. Decreased partial thromboplastin time that is corrected by mixing 1:1 with fresh-frozen plasma

2. Decreased partial thromboplastin time that is not corrected by mixing 1:1 with fresh-frozen plasma

3. Prolonged partial thromboplastin time that is corrected by mixing 1:1 with fresh-frozen plasma

4. Prolonged partial thromboplastin time, not corrected by mixing 1:1 with fresh-frozen plasma

Prolonged partial thromboplastin time, not corrected by mixing 1:1 with fresh-frozen plasma

1. APS is characterized by hypercoagulability, frequent miscarriages, thrombocytopenia, and a false-positive rapid plasma reagin test result.

2. APS is characterized by a prolonged PTT that is not corrected by mixing 1:1 with fresh-frozen plasma and can be detected by the presence of specific serum autoantibodies, including anticardiolipin or anti–beta-2-glycoprotein

300

A 53 year old man with atrial fibrillation (increased risk of stroke) is started on warfarin for prophylaxis. 2 days later, he develops microthrombi throughout his body. 

Why did this most likely occur?

1. Drug-drug interaction with metoprolol (a beta blocker used for patients with atrial fibrillation) rendered the warfarin ineffective

2. Lack of Heparin bridging prior to starting the patient on warfarin

3. Anti-Warfarin antibodies were produced, severely reducing warfarin’s anticoagulation effect

4. His atrial fibrillation worsened, causing the microthrombi to form

Lack of Heparin bridging prior to starting the patient on warfarin

1. Activated Protein C inhibits Factors Va and VIIIa

2. Hypercoagulation: Protein C half life < Factors II and X; Protein C depletes faster than Factors II and X, leading to hypercoagulability 

3. Starting with Heparin before transitioning to warfarin enables factors IIa and Xa to be inhibited first. Transitioning a patient to warfarin after this will prevent the problem of protein C being depleted before factors IIa and Xa, preventing the patient from entering a hypercoagulable state

400

A 45-year-old woman is brought to the emergency department because of confusion, fever, and lethargy. Temperature is 38.3°C (101°F). On examination, the patient is disoriented and appears jaundiced. Laboratory studies show:

Hemoglobin:  8 g/dL (↓)

Platelet count:  30,000/mm³ (↓)

Creatinine:  2.2 mg/dL 

Blood urea nitrogen:  29 mg/dL

Lactate dehydrogenase:  350 U/L

Total bilirubin:  2.8 mg/dL (↑)

Indirect bilirubin:  2.0 mg/dL (↑)

A peripheral blood smear is shown.

Which of the following is the underlying cause of this patient’s most likely condition?

1. Antibodies directed against platelets

2. Inherited disorder of hemoglobin

3. Decreased activity of von Willebrand factor protease

4. Mutations affecting the erythrocyte membrane

5. Viral infection of hepatic cells

Decreased activity of von Willebrand factor protease

1. TTP is characterized by a classic pentad of central nervous system symptoms, renal insufficiency, fever, thrombocytopenia, and microangiopathic hemolytic anemia and schistocytes on PBS.

2. TTP is the result of a decreased amount or an absence of the normal vWF protease ADAMTS 13.

500

A 6-year-old boy is brought to the pediatrician by his father with easy bruising of his legs and heavy bleeding when he brushes his teeth. The patient has recurrent otitis media and has been treated with multiple courses of antibiotics, including ceftriaxone. Laboratory tests show prothrombin time of 22 seconds, international normalized ratio of 1.5, and activated partial thromboplastin time of 47 seconds.

Which of the following mechanisms is most likely inhibited in this patient?

1. Activation of antithrombin III

2. Carboxylation of glutamic acid residues

3. Cross-linking of fibrin polymers

4. Hydroxylation of proline residues

Carboxylation of glutamic acid residues

1. Vitamin K deficiency presents with easy bruising and a propensity for bleeding, along with an increased PT and PTT.

2. Vitamin K deficiency can occur after the prolonged use of broad-spectrum antibiotics, particularly cephalosporins. Vitamin K is required for the carboxylation of glutamic acid residues, a key event to support the function of factors within the clotting cascade

Leukemias/Lymphomas/Myelodysplastic

100

A 51-year-old man comes to the physician because of a 2-month history of multiple lumps on his body that increase and decrease in size. He also describes unintentional weight loss and intermittent night sweats. His temperature is 38.1° C (100.6° F). Examination reveals diffuse nontender lymphadenopathy. Results of a lymph node biopsy are shown.



Which of the following oncogenes and translocations is associated with this type of lymphoma?

A) BCL-2; t(14;18)

B) BCR-ABL; t(9:22)

C) Cyclin D1-heavy chain Ig gene; t(11:14)

D) PML-RARA; t(15;17)

E) c-myc; t(8,14)

A) BCL-2; t(14;18)

Follicular lymphoma may present with painless lymphadenopathy. Approximately 20% present with “B symptoms.”

Follicular lymphoma expresses BCL-2 as a result of t(14;18) translocation.

200

An 86-year-old man comes to the clinic because of a 4-month history of increasing fatigue, a 6.8-kg (15-lb) unintentional weight loss, and an uncomfortable fullness in the left upper quadrant. On examination, there is conjunctival pallor and no lymphadenopathy. A left upper quadrant mass is felt on palpation. A complete blood cell count shows:

WBC count:  14,000/mm3 (high)

Hemoglobin:  9 g/dL (low)

Hematocrit:  30% (low)

Platelet count:  500,000/mm3 (high)

A peripheral blood smear shows dacryocytes and the structure shown in the center of the figure. Several attempts at bone marrow aspiration were unsuccessful.

Which of the following processes is most likely associated with this patient’s condition?

A) Aplastic anemia

B) Asplenia

C) Hemoglobin clumping

D) Intravascular hemolysis

E) Myelofibrosis

E) Myelofibrosis

Primary myelofibrosis is a myeloproliferative disorder in which bone marrow is gradually replaced by collagen fibers. It may present with fatigue, anemia, splenomegaly, teardrop-shaped RBCs, leukoerythroblastic reaction on smear, and an unsuccessful attempt at obtaining bone marrow aspiration.

In myelofibrosis, compensatory extramedullary hematopoiesis may take place in the liver, spleen, and lymph nodes. Dacryocytes, or teardrop-shaped RBCs, are the result of physical damage sustained from the squeezing of RBCs out of increasingly fibrotic bone marrow.

300

A 65-year-old male comes to the physician with a 6-month history of intermittent headaches. In the past few weeks, the patient has had two brief episodes of vision loss in his right eye lasting approximately 10 to 15 seconds each. Physical examination is normal. A complete blood cell count shows a WBC count of 9,300/mm3, hemoglobin of 12.5 g/dL, and a platelet count of 1,020,000/mm3. A blood smear is shown.



This patient most likely has which of the following disorders?

A) Acute promyelocytic leukemia

B) Chronic myelogenous leukemia

C) Essential thrombocythemia

D) Polycythemia vera

E) Primary myelofibrosis

C) Essential thrombocythemia

Essential thrombocytosis is characterized by very high platelet counts and the absence of other causes of high platelets (eg, infection, bleeding, or other myeloproliferative disorders).

Patients with essential thrombocythemia have high platelet counts due to excessive platelet production rather than prolonged platelet survival.

400

A 25-year-old man comes to the emergency department because of abdominal pain and swelling. He denies having a bowel movement for the past week. Imaging shows an abdominal mass. Histopathologic examination of a specimen obtained on biopsy of the mass reveals sheets of highly mitotic, basophilic lymphocytes surrounding clear zones of macrophages.

Which of the following is the most likely underlying cause of these findings?

A) Acute lymphoblastic lymphoma

B) Burkitt lymphoma

C) Follicular lymphoma

D) Multiple myeloma

E) Polycythemia vera

B) Burkitt lymphoma

Burkitt lymphoma is a highly aggressive form of non-Hodgkin lymphoma, and common symptoms include anorexia, constipation, and abdominal pain. Biopsy often reveals highly mitotic, basophilic lymphocytes surrounding clear zones of macrophages.

500

A 19-year-old male comes to the office with a 1-week history of fever to 38.6°C (101.5°F), weakness, and fatigue. Physical examination shows pale conjunctivae and diffuse petechiae across his trunk. Complete blood count shows the following:

Hemoglobin:  7 g/dL (↓)

Hematocrit:  21% (↓)

Reticulocyte count:  0.1% (normal 0.5%-1.5%) (↓)

Platelet count:  50,000/mm3 (↓)

Leukocytes:  17,300/mm3 (↑)

Neutrophils:  44% (↓)

Eosinophils:  2%

Lymphocytes:  46% (↑)

Monocytes:  6%

A chest x-ray is shown. A biopsy of the mass seen on the x-ray reveals cells with light-blue, scanty, and nongranular cytoplasm.

Which of the following is the most likely diagnosis?

A) B-cell acute lymphoblastic lymphoma

B) Chronic lymphocytic leukemia

C) Follicular lymphoma

D) Hodgkin disease

E) T-cell acute lymphoblastic lymphoma

E) T-cell acute lymphoblastic lymphoma

T-cell acute lymphoblastic lymphoma may present with signs and symptoms of anemia, thrombocytopenia, leukocytosis, and mediastinal mass. Biopsy of the mass would show cells with light-blue, scanty, and nongranular cytoplasm, which represents lymphoblasts.

Favorite Snacks

100

What is Adam's favorite snack?

Apples

Oreos

Doritos (Purple bag)

Pistachios

Apples

200

Emad's favorite snack lately:

A) Ritz crackers (roasted vegetable kind)

B) Lotus Bischoff cookies

C) Dark chocolate M&Ms

D) String cheese

E) Strawberries

C) Dark chocolate M&Ms

300

Athina Jaffers Favorite snack:

A. Extra Toasty Cheese itz

B. Jell O Pudding

C. Herrs Kettle Salt and Vinegar chips

D. Tastycakes powdered mini donuts

E. tru fru white and dark chocolate covered rasberries

E