Chapter 14 Pathology (pgs. 639-665) (No Answer Document) Match the following (1-3): a) MCV b) MCH c) MCHC 1) Average content of hemoglobin per red cell in picograms. 2) Average volume of RBCs in femtoliters (fL). 3) Average concentration of hemoglobin in a given volume of packed red cells, grams per deciliter. 4) T/F Iron in hemoglobin can be recaptured if it is lost into tissue space. 5) T/F Iron in hemoglobin can be recaptured if it is lost into the gut. 6) T/F Extravascular hemolysis can lead to organomegaly. 7) Which of the following doesn’t cause splenomegaly? a. Extravascular hemolysis b. Intravascular hemolysis c. Hereditary spherocytosis d. Sequestration crises e. Sickle cell anemia 8) What is the function of haptoglobin? a. Bind vitamin B12 for absorption b. Promote iron excretion in urine c. Bind hemoglobin and prevent it from be excreted in urine d. Destroy red cells e. Parvovirus mechanism for aplastic anemia 9) T/F Intravascular anemia causes hemoglobinemia and hemosiderinuria. 10) T/F Heme breakdown causes the formation of bilirubin as a product which is the main cause of jaundice. 11) Spectrin heads bind with which of the following? a. Other spectrin heads b. Actin c. Glycophorin A d. Protein 4.1 e. Spectrin tails 12) Spectrin and band 3 bind to each other via? a. Ankyrin and band 4.1 b. Ankyrin and band 4.2 c. Actin and protein 4.1 d. Ankyrin and protein 4.1 e. Actin and glycophorin A 13) T/F The most common cause of hereditary spherocytosis is mutations to actin, protein 4.1, and glycophorin A. 14) Spherocytes have an increased MCHC because? a. Loss of Ca++ and H2O b. Loss of Na+ and H2O c. Loss of K+ and Na+ d. Loss of K+ and H2O e. Loss of K+, Na+, and H2O 15) A patient has a blood smear done and dark nuclear remnants in his red cells appear and you diagnose him with hereditary spherocytosis, what are those nuclear remnants called? a. Heinz bodies b. Alpha chain aggregates c. Howell Jolly bodies d. Hemoglobin Barts e. HBH 16) What is the correct order in glutathione metabolism from G6P to oxidant reduction? a. G6PD to NADP to Glutathione b. G6PD to NAD to Glutathione c. Glutathione to NAD to G6PD d. Glutathione to NADP to G6PD e. NAD to G6PD to Glutathione 17) A man with excess oxidant stress secondary to an infection by pneumococcus pneumoniae has inclusions caused by sulfhydryl cross linking of globin chains, these are called what? a. Heinz bodies b. Howell Jolly bodies c. Alpha chain aggregates d. Hemoglobin Barts e. HBH 18) G6PD deficiency is? a. Autosomal recessive b. Autosomal dominant c. Y-linked d. Caused by point mutations e. X-linked 19) T/F G6PD minus has the shortest half-life. 20) T/F Fava beans are detrimental to G6PD deficient individuals. 21) A patient with heterozygous sickle cell anemia (HbA and HbS) is? a. 30% HbA and 70% HbS b. 70% HbA and 30% HbS c. 60% HbA and 40% HbS d. 40% HbA and 60% HbS e. 50% HbA and 50% HbS 22) A patient comes in with dehydration and sickling of his red cells, you do a genetic analysis and determine he doesn’t have the more severe HbSS but instead he has HbSC. HbS and HbC are caused by the following amino acid exchanges respectively: a. Valine for glutamine and lysine for glutamine b. Valine for glutamate and lysine for glutamine c. Valine for glutamate and lysine for glutamate d. Arginine for glutamine and glycine for glutamate e. Arginine for glutamate and glycine for glutamate 23) T/F Sickling occurs under oxygenated conditions. 24) After an amniocentesis and genetic analysis of a baby you determine the baby will have sickle cell anemia that will present after around 6 months. Why did you make this claim? a. The baby is protected by maternal antibodies that destroy sickle cells early b. HbC protect the baby c. Surfactant in the infant is adequate to overcome sickling events d. The infant has enough normal red cell progenitors that can prevent sickling e. The infant has HbF which protective against sickling 25) T/F Sickle cells cause microvascular occlusions because of inflammation, increased surface adhesion expression, and decreased Hb binding of NO. 26) To narrow down possible diseases you do a skull x-ray that shows a crew-cut calvarium and prominent cheek bones, which of the following could MOST likely cause this other than sickle cell anemia? a. Hereditary Spherocytosis b. G6PD deficiency c. Aplastic anemia d. Myelophthisic anemia e. B-thalassemia 27) While karyotyping a patient with alpha thalassemia you look for defects for alpha chains on which of the following? a. Two identical genes on chromosome 16 b. One gene on chromosome 11 c. One gene on chromosome 15 d. One gene on chromosome 16 e. Two identical genes on chromosome 11 28) Beta + defects in beta-thalassemia patients is most commonly caused by: a. Splicing mutations b. Insertions c. Operon mutations d. Promoter region mutations e. Chain terminator mutations 29) Beta thalassemia major which is homozygous was diagnosed in a newborn (beta0/beta-0 variant), which of the following insoluble aggregates are causing red cell membrane destruction, ineffective erythropoiesis, and extramedullary hematopoiesis? a. Beta aggregates b. HbH c. Hemoglobin Barts d. Alpha aggregates e. Gamma aggregates 30) A patient suffering from beta thalassemia was given blood transfusions and he started forming iron deposits in his organs because of the following unknown etiologic effect? a. DMT1 overexpression b. Decreased hepcidin c. Increased hephaestin d. Decreased ferroportin e. Increased ceruloplasmin 31) Alpha thalassemia is less likely to cause hemolysis and ineffective erythropoiesis because? a. Alpha aggregates are less soluble and condense to the center of the red cell b. Beta chains are more soluble c. Beta chains are less soluble d. Gamma chains create insoluble aggregates e. Alpha chains are very soluble 32) T/F In Asians the alpha thalassemia trait is (a/a),(-/-) and they are more likely to have thalassemia than Africans. 33) T/F HbH is a tetramer of beta chains and occurs when 1 alpha gene is defective. 34) A fetus in the third trimester is suffering from Hydrops Fetalis and you as a physician immediately start blood transfusions and recommend a bone marrow transplant when possible. How many genes are missing/mutated? a. Two alpha genes b. Three beta genes c. Four beta genes d. Four alpha genes e. Three alpha genes 35) The leading cause of death in Paroxysmal Nocturnal Hemoglobinuria (PNH) is? a. Iron overload b. Jaundice c. Megaloblastic anemia d. Immune Gastritis e. Thrombosis 36) T/F A direct Coombs antiglobulin test requires commercial red blood cells and a patient’s blood serum. 37) Warm antibody type immunohemolytic anemia is most commonly caused by which immunoglobulin? a. IgA b. IgD c. IgE d. IgG e. IgM 38) Vitamin B12 deficiency causes pernicious anemia and megaloblastic anemia mainly because of? a. Autoimmune gastritis b. Folate deficiency c. Homocysteine increase d. Methylmalonate increase e. Gastric carcinoma 39) What is intrinsic factor used to absorb and what part of the intestinal tract is the vitamin/mineral absorbed? a. Folic acid/jejunum b. Folic acid/duodenum c. B12/ileum d. B12/jejunum e. B12/duodenum 40) Even though pernicious anemia occurs due to primarily T cell damage to parietal cells, associated type III autoantibodies do what? a. Destroy intrinsic factor b. Destroy transcobalamin II c. Affect the gastric proton pump d. Inhibit the binding of B12 and intrinsic factor e. Inhibit internalization of B12-intrinsic factor complex by enterocytes 41) A patient presents with fundic gland atrophy, intestinalization, and spinal cord demyelination because of his poor diet which has caused a deficiency of? a. B12 b. Folate c. Iron d. K+ e. Methionine synthase 42) Which of the following won’t be fixed by parenteral administration of B12 in a deficient patient? a. Anemia b. Vascular disease c. Neurological symptoms d. Atrophic glossitis e. Gastric atrophy 43) Folate is not involved in which of the following? a. dTMP formation b. Purine synthesis c. Methylmalonate metabolism d. Homocysteine metabolism e. FH4 production 44) A patient comes in with neurological issues and megaloblastic anemia, which of the following will help fix the megaloblastic anemia but not the neurological issues? a. Iron b. Pteroylmonoglutamic acid c. B12 d. Cobalamin e. Ceruloplasmin Match the following (45-49) (some may be used once, more than once, or not at all): a) b) c) d) e) f) g) Transferrin Ferritin Hephaestin DMT1 Ferroportin Nonheme Iron Heme Iron 45) Transports Fe3+ in the blood. 46) Allows iron to be taken into enterocytes from the lumen of the proximal duodenum. 47) Is storage form of iron. 48) Inhibited by hepcidin on not only enterocytes but also macrophages. 49) Animal Hb form of iron. 50) T/F Beta Thalassemia causes primary hemochromatosis. 51) A patient who has been drinking a lot of tea has developed microcytic hypochromic anemia and koilonychia because of iron deficiency, you advise the patient to increase their intake of? a. Tannates b. Carbonates c. Oxalates d. Citric acids e. Phosphates 52) A patient with drastically depleted ferritin iron levels comes to Westmoreland Hospital. You being the brightest intern suspect the underlying cause to most likely be? a) Chronic bleeding b) Menstruation c) Dietary insufficiency d) Increased Hepcidin e) High tea consumption 53) Anemia with chronic disease is most likely caused by which cytokine? a. IL-10 b. IL-12 c. IL-7 d. IL-1 e. IL-6 54) T/F In aplastic anemia a dry tap occurs, fat/fibrotic tissue replaces marrow, and splenomegaly is missing. 55) A patient’s blood smear shows tear drop shaped red cells, and you diagnose the patient with? a. Polycythemia b. Alpha Thalassemia c. Hereditary Spherocytosis d. Myelophthisic anemia e. Aplastic anemia 56) Polycythemia Vera is a? a. Relative polycythemia associated with high erythropoietin. b. Relative polycythemia associated with low erythropoietin. c. Absolute polycythemia associated with high erythropoietin. d. Absolute polycythemia associated with low erythropoietin.