2012 module exam Flashcards
Which of the following is an iron storage form found in all cells?
a. ferritin
b. ferroportin
c. transferrin
d. hepcidin
a. ferritin
An iron storage complex stained with Prussian blue
a. transferrin
b. hemosiderin
c. ferritin
b. Hemosiderin
How can lead cause anemia?
a. By inhibitingaminolevulinic acid synthase
b. By inhibiting aminolevulinic acid dehydratase
b. By inhibiting aminolevulinic acid dehydratase
What would be increased in a patient who developed megaloblastic anemia after a gastrectomy?
Methylmalonic acid
Transcobalamin III (aka intrinsic factor) is secreted from stomach, i.e. gastrectomy →decreased IF →decreased absorption of B12 →decreased methylmalonyl-CoA mutase rxn → increased methylmalonyl-CoA & decreased succinyl-CoA
What is the correct sequence of hematopoiesis in the fetus?
a. Yolk sac, liver, placenta, bone marrow
b. Yolk sac, spleen, lymph nodes, bone marrow
c. Bone marrow, liver, spleen, yolk sac
d. Yolk sac, spleen, lymphoid organs, bone marrow
b. Yolk sac, spleen, lymph nodes, bone marrow
A 20-year old male was in a car accident. He has a lacerated liver and multiple fractures. He lost a lot of blood and his Hb was 50 g/L. He was given 20 units of packed cells. What complication would most commonly occur in this patient?
a. Hyperkalemia
b. Hypokalemia
a. Hyperkalemia
(Extended blood storage also reduces flippase activity →PS exposure on outer leaflet →coagulation & RBC sequestration)
Which of the following is an initial test in the workup of a patient with a suspected case of hemolytic anemia?
a. Reticulocyte count
b. Osmotic fragility test
c. G6PD enzyme levels
d. Hemoglobin electrophoresis
a. Reticulocyte count
Which of the following will result in agglutination in a cross-matching reaction?
a. Mixing the donor’s RBCs with the recipient’s serum
b. Mixing the donor’s serum with the recipient’s RBCs
a. Mixing the donor’s RBCs with the recipient’s serum
(packed red cells contain no/little serum and therefore they will not react with recipient’s RBCs, this is why the second option is wrong)
What would you give a patient who suffers from hepatic encephalopathy with prolonged PT and APTT?
a. Platelets
b. Fresh frozen plasma
c. PRBC
b. Fresh frozen plasma
A 45-year old female who has been on chemotherapy for 2 days developed nose bleeds. What would you give her?
a. Platelets
b. Fresh frozen plasma
c. PRBC
a. Platelets
A 25 year old male has been in a road-traffic accident. He lost a significant amount of blood. What would you give him?
a. Platelets
b. Fresh frozen plasma
c. PRBC
c. PRBC
Which of the following is an intravascular hemolytic anemia? a. Beta-thalassemia major b, Autoimmune hemolytic anemia c. Thrombotic thrombocytopenic purpura d. Hereditary elliptocytosis e. Hereditary spherocytosis
c. Thrombotic thrombocytopenic purpura
What hemoglobin might you detect in a newborn expected with alpha-thalassemia trait? a-Hb Gower 1 b-Hb Bart c-Hb Portland d-HbH e-HbE
b-Hb Bart
(Hb Barts (γ4) is usually found in 4 gene deletion α‐thalassemia and causes hydrops fetalis, but can be seen in α‐thalassemia trait (she said in the lecture to memorize it). HbH (β4) is seen with 3 gene deletion)
Its deficiency leads to severe hemolytic crisis when certain medications are taken A. NADPH B. NADP C. Glucose 6-phosphate dehydrogenase D. aminolevulinic acid dehydrase E. glucose 6-phosphate F. 2, 3-bisphosphoglycerate
C. Glucose 6-phosphate dehydrogenase
Its production in the erythrocyte affects the binding affinity of hemoglobin for O₂ A. NADPH B. NADP C. Glucose 6-phosphate dehydrogenase D. aminolevulinic acid dehydrase E. glucose 6-phosphate F. 2, 3-bisphosphoglycerate
F. 2, 3-bisphosphoglycerate
Its low concentration in the RBC protects against malaria A. NADPH B. NADP C. Glucose 6-phosphate dehydrogenase D. aminolevulinic acid dehydrase E. glucose 6-phosphate F. 2, 3-bisphosphoglycerate
A. NADPH
Lead binds to it resulting in anemia in lead poisoning A. NADPH B. NADP C. Glucose 6-phosphate dehydrogenase D. aminolevulinic acid dehydrase E. glucose 6-phosphate F. 2, 3-bisphosphoglycerate
D. aminolevulinic acid dehydrase
What causes the release of O₂ from Hb to peripheral tissue?
a- Acidification
b- Biconcave shape of RBCs
a- Acidification
H+
What protects factor VIII from degradation?
a. plasmin
b. antithrombin
c. vWF
d. factor XI
c. vWF
At which phase do blood islets form?
a. liver phase
b. Yolk sac phase
c. BM phase
b. Yolk sac phase
What is the product of vitamin K dependent synthesis?
Gla (γ‐carboxyglutamate)
Ab against which cytokine will help in treating anemia of chronic disease?
a- IL-6
b- IL-10
c- IL-11
a- IL-6
Which hemoglobin would you find in a newborn that would decrease soon after birth? a- HbF b- HbA2 c-Hb Gower 1 d-Hb Bart
A- HbF
A 76-year old male with ischemic heart disease with symptoms of anemia, blood in stool. PT, APTT, and platelet count is normal. What is the most likely diagnosis?
a- Platelet dysfunction
b- Aspirin therapy
b- Aspirin therapy
A 3-year old girl was brought by her mother to the ER with bleeding out of her venipuncture sites and prolonged bleeding after a tooth extraction. Her mom mentioned that a family member has a similar problem. Her APTT and PT were normal. What is the most likely diagnosis?
a. Von Willebrand Disease
b. factor 9 deficiency
c. Platelet dysfunction
a. Von Willebrand Disease
What are the cells that you see in the pale region of a thrombus?
a- Dead RBC’s and vWF
b- Fibrin + platelets
c- factor 9 & 10
b- Fibrin + platelets
What best describes secondary hemostasis?
a- Both extrinsic and intrinsic pathways produce factor X
b- vWF plays an important role in this process
a- Both extrinsic and intrinsic pathways produce factor X
A 17-year old boy presents with fever, sore throat, difficulty swallowing, and generalized malaise. Examination reveals a whitish membrane covering the pharynx. A blood smear reveals the presence of atypical mononuclear cells. What is the causative agent?
a. CMV
b. EBV
c. HIV
b. EBV
(Corynebacterium diphtheriae-> causes white, thick covering in the back of the throat. It can lead to difficulty breathing)
A patient with splenomegaly. PBS shows increased mature lymphocytes and smudge cells. What is the diagnosis?
a. Chronic lymphocytic leukemia
b. thalassemia major
c. G6PD
a. Chronic lymphocytic leukemia
Compared to high grade lymphoma, what is the characteristic of indolent lymphoma?
Slowly progressing but more difficult to cure
Which of the following protects vitamin B12 from denaturation in the stomach?
a. Haptocorrin
b. Gastric intrinsic factor
c. Cubulin
a. Haptocorrin
transcobalamin I=R protein
What binds vitamin B12 in the duodenum?
a. Transcobalamin I
b. Gastric intrinsic factor
c. Cubulin
b. Gastric intrinsic factor
transcobalamin III
The immune destruction of which of the following results in pernicious anemia?
a. parenchymal cells
b. parietal cells
b. parietal cells
What is methemoglobin?
a. The reduced heme is combined with carbon monoxide
b. Heme contains iron in the reduced form (Fe2+)
c. Hemoglobin with Fe³⁺ in the heme
c. Hemoglobin with Fe³⁺ in the heme
Which of the following is true regarding MGUS?
a. It is not associated with clinical symptoms
b. It is a benign condition
c. Plasma cells are found at a concentration of more than 10% in the bone marrow
d. 50% progress to plasma cell myeloma
a. It is not associated with clinical symptoms
Which of the following is true regarding primary amyloidosis?
a. The deposition of proteins extracellularly in an abnormal fibrilliar form
b. The deposition of proteins intracellularly in a fibrilliar form
a. The deposition of proteins extracellularly in an abnormal fibrilliar form
Which of the following confirms the diagnosis of multiple myeloma?
a- Bence-jones protein in urine
b- Reduced bone density
c- Low calcium levels
d- Polyclonal increase in gamma globulins
e- Increased serum protein concentration
a- Bence-jones protein in urine
A patient presented with unexplained weight loss and lymphadenopathy. He was found to have the t(14, 18) translocation. What is the diagnosis?
Follicular lymphadenopathy
Which is a pro-drug that causes hemorrhagic cystitis?
a. Imatnib
b. Cyclophosphamide
c. Methotrexate
b. Cyclophosphamide
Which of the following a tyrosine kinase inhibitor?
a. Methotrexate
b. Cyclophosphamide
c. Imatnib
c. Imatnib
What is a characteristic of alkylating antineoplastic drugs used in the treatment of hematological malignancies?
a. Disrupt protein folding
b. Act on proliferating and non-proliferating cells
c. Act on proliferating cells only
b. Act on proliferating and non-proliferating cells
Which of the following is a dihydrofolate reductase inhibitor?
a. Cyclophosphamide
b. Imatnib
c. Methotrexate
c. Methotrexate
A patient complains of fever and unexplained weight loss. He also has lymphadenopathy. Lymph node biopsy shows a follicular pattern. Which test would you use to diagnose this condition?
a- Fluorescence in-situ hyperdization
b- Cytochemical staining
b- Peripheral blood smear
a- Fluorescence in-situ hyperdization
A 60-year old male complains of weight loss. Examination reveals lymphadenopathy. A biopsy of the lymph node shows a nodular pattern with bi-nucleated cells. What is the causative agent?
a) HIV
b) Hepatitis C
c) EBV
d) HTLV-1
c) EBV
(This is a case of the nodular sclerosing type of Hodgkin’s lymphoma (HL) with the characteristic Reed-Sternberg cells (CD15+, CD30+). EBV is associated with BOTH Hodgkin’s lymphoma and Burkitt lymphoma. HIV, however, is associated with some NHLs)
A 65-year old female complains of fever and night sweats. Imaging reveals the presence of mediastinal lymph node enlargement. A biopsy reveals the presence of bi-nucleated cells with prominent nucleoli that are CD15 and CD30 positive. What is the most likely diagnosis?
a) Follicular lymphoma
b) Hodgkin’s lymphoma
c) Chronic lymphocytic leukemia
d) Acute lymphocytic leukemia
b) Hodgkin’s lymphoma
What is the function of the PML/RARA fusion protein?
Stimulates the proliferation of the promyelocytes
PML-RARA inhibits differentiation of promyelocytes and maintains it at an active proliferating state
A patient presented with thrombosis in the deep veins of his right leg following a long flight. His CBC reveals grossly elevated platelets (1350) with normal Hb and WBC count. What would you expect to find in the bone marrow of this patient?
a. Megakaryotic hyperplasia
b. Bone marrow hypoplasia
a. Megakaryotic hyperplasia
What is the consequence of the BCR-ABL translocation?
a. Makes more growth factors
b. Makes the cells more sensitive to growth factors
b. Makes the cells more sensitive to growth factors
A patient has a transfusion dependent anemia. His CBC showed pancytopenia. His PBS showed macrocytic and hypolobated neutrophils. What is the diagnosis? a- aplastic anemia b-hyperthyroidism c-megaloblastic anemia d-myelodysplastic syndrome
d-myelodysplastic syndrome
What is an anti-bacterial peptide released by neutrophils?
A) Lysozyme
B) Major basic protein
C) Defensins
C) Defensins
What is used by granulocytes to opsonize bacteria? A) Complement fragments B) Immunoglobulins D) Fc receptors E) Defensins
D) Fc receptors
A 45-year old male presents with epistaxis and fatigue. His CBC shows pancytopenia. The PBS shows circulating immature cells. What is the pathogenesis of this condition?
Acute leukemic infiltration
A patient presented with fever, petechial, shortness of breath, and fatigue. He has splenomegaly. His PBS shows circulating immature cells with intracytoplasmic rods. What is the most likely diagnosis?
a) Follicular lymphoma
b) Hodgkin’s lymphoma
c) Chronic lymphocytic leukemia
d) Acute myeloid leukemia
d) Acute myeloid leukemia
What is the most common abnormality found in MDS?
a. Hypercellular bone marrow and reduced cell count
b. Normocellular bone marrow and reduced cell count
c. Necrotic bone marrow and reduced cell count
d. Fibrotic bone marrow and reduced cell count
e. Hypocellular bone marrow and reduced cell count
a. Hypercellular bone marrow and reduced cell count
Which of the following is an antibacterial substance found in eosinophilic granules?
a. Major basic protein
b. Cationic protein
a. Major basic protein
What is the function of the ETO/AML1 fusion protein?
a) transcription factor
b) TNF
c) tyrosine Kinase inhibitor
a) transcription factor
A person complaining of severe asthmatic attack. His WBCs are elevated. What would you expect to be elevated in the differential count?
a. Promyelocytes
b. Neutrophils
c. Blasts
d. Eosinophils
d. Eosinophils
A 27-year old female has deep vein thrombosis in the lower limb and she has factor V Leiden mutation. What does she suffer from?
a. More sensitive to protein C
b. Resistance to protein C
b. Resistance to protein C
What is an antibacterial substance found in neutrophilic granules?
Lactoferrin
why not Defensins?
A patient present with a petechial rash and fatigue. CBC reveals reduced platelets and Hb. PBS reveals fragmented cells. His coagulation profile is normal. What is the most likely diagnosis?
A) antiphospholipid syndrome
B) DIC
C) TTP
C) TTP
Thrombotic thrombocytopenic purpura
A 35-year old woman developed a pulmonary thromboembolism. She has a history of a still-birth at 6 months. Her PT and APTT are normal and she is positive for lupus anticoagulant. What is the most likely diagnosis?
a) vWF deficiency
b) DIC
c) vitamin k deficiency
d) antiphoshphilipid syndrome
d) antiphoshphilipid syndrome
A 19-year old was brought by her mother to the hospital for a bleeding disorder. What would indicate that her bleeding is due to a platelet problem and not a coagulation problem?
a. Joint swelling
b. Having received fresh frozen plasma in the past
c. Bleeding several hours after a tooth extraction
d. Excessive menstrual bleeding
d. Excessive menstrual bleeding
A patient with a history of thromboembolism with prolonged APTT not corrected with normal plasma. What is the diagnosis?
a. Factor V deficiency
b. Lupus anticoagulant
b. Lupus anticoagulant
What causes thrombophilia in a patient with factor V Leiden mutation?
a. It becomes resistant to protein C
b. It becomes more sensitive protein C
a. It becomes resistant to protein C
A premature neonate presents with prolonged bleeding from his umbilical stump. He has prolonged PT and APTT. What is the most likely cause?
a) vWF deficiency
b) DIC
c) vitamin k deficiency
c) vitamin k deficiency
Which protease starts the common pathway?
a. Xa
b. XIIa
c. IXa
a. Xa
A 16-year old girl suffers from recurrent easy bruising. She has hyper extensible skin and joints. What might be responsible for her condition?
a) vWF deficiency
b) DIC
c) Vitamin k deficiency
d) Blood vessel disorder
d) Blood vessel disorder
Which molecules are responsible for platelet aggregation?
a. plasmin
b. ADP and TBXA2
c. antithrombin
b. ADP and TBXA2
A 31-year old female was admitted to the hospital with a gram-negative bacterial infection. Upon examination, she was found to be septic. She had petechial rashes and easy bruising. CBC shows anemia and low platelets. PBS shows RBC fragments. Her D-dimer is elevated. What is the most likely diagnosis?
a) vWF deficiency
b) DIC
c) vitamin k deficiency
b) DIC
What would be the consequence of a mutation in the 3’UTR of the prothrombin gene?
a. increase activity of factor V leiden
b. Stabilization of Prothrombin mRNA
c. Protein C becomes inactive
d. DNA of antithrombin becomes more active
b. Stabilization of Prothrombin mRNA
What causes the abnormal migration of HbS in sickle cell anemia?
a. Increase charge
b. Decrease charge
c. Lower volume
b. Decrease charge
What level of factor VIII would you expect to find in a person with severe hemophilia A?
a. More than 30%
b. Less than 1%
b. Less than 1%
A 56-year old female presented with a sudden onset of acute dyspnea. Shortly after that, she went into cardiac arrest. The autopsy shows a saddle thromboembolism. Which of the following conditions is the most likely cause?
a. Heterozygous Factor V Leiden mutation
b. Protein C deficiency
c. Thrombocytopenia
a. Heterozygous Factor V Leiden mutation
A 23-year old male patient in the intensive care unit has easy bruising and bleeding from venipuncture sites. He has low platelets and fibrinogen. What is this condition?
a) vWF deficiency
b) DIC
c) vitamin k deficiency
d) antiphoshphilipid syndrome
b) DIC
How does heparin work?
a. Inhibits platelets cyclooxygenase
b. Accelerates the action of anti-thrombin III
b. Accelerates the action of anti-thrombin III
What is the most important thing for the RBC to withstand the mechanical sheer force of moving through small blood vessels?
a- G6PD enzyme present in RBC
b- Integral membrane protein noncovalent attachment to cytoskeleton
c- hemoglobin distributed evenly across the cell
b- Integral membrane protein noncovalent attachment to cytoskeleton
Aspirin is given in low doses to prevent thrombosis. What is the mechanism of action of aspirin?
a. Inhibits platelets cyclooxygenase
b. Accelerates the action of anti-thrombin III
a. Inhibits platelets cyclooxygenase
A transfusion dependent MDS patient is on regular blood transfusions. He presents with symptoms of heart failure. Upon examination, his serum ferritin was found to be 4500 µg/L. What would be administered to treat him?
A) deferasirox
B) deferoxamine
C) deferiprone
B) deferoxamine
A 15-year old female suffers from long standing anemia. Her peripheral blood smear shows Howell-jolly bodies. What is the most likely diagnosis?
a. G6PD
b. Sickle cell anemia
c. spherocytosis
d. eliptocytosis
b. Sickle cell anemia
(Howell-Jolly bodies, which are basophilic nuclear remnants in RBCs, are normally removed by splenic macrophages, i.e. their presence indicate hyposplenia/asplenia which is characteristic of sickle cell anemia)
Which are associated with gangliosides in RBCs?
a) Indication of cell death
b) Regions of low lipid fluidity
c) lipid interactions with membrane proteins
d) Lipid clusters with covalent bonds
b) Regions of low lipid fluidity
A 28-year old female presents with fatigue, dyspnea, and shortness of breath. Her CBC shows increased reticulocyte and low Hb. PBS shows spherocytic morphology. The direct antiglobulin test is positive. What is the most likely diagnosis?
a. Immune mediated hemolysis
b. spherocytosis
c. G6PD
a. Immune mediated hemolysis (auto-immune hemolytic anemia)
(Spherocytes can be seen in hereditary spherocytosis (ankyrin mutation) and autoimmune hemolytic anemia, which occurs in CLL)
Which molecule links glucose metabolism with hemoglobin affinity for oxygen?
a. 1, 3-bisphosphoglycerate
b. 1, 3-phosphoglycerate
c. 2, 3-bisphosphoglycerate
d. 2, 3-phosphoglycerate
c. 2, 3-bisphosphoglycerate
What results from the inactivation of the pentose phosphate pathway?
a. Acidification
b. utilization of O2 in RBC
c. Oxidative damage
b. Oxidative damage
What causes the shift of the hemoglobin saturation curve from left to right?
a. Acidification
b. Oxidative damage
a. Acidification
