1. The process of formation and development of blood cells is termed: a. Hematopoiesis c. Hematocytometry b. Hematemesis d. Hematorrhea

Regulation of the hematopoietic microenvironment is provided by the: a. Stromal cells and growth factors b. Hematopoietic stem cells c. Liver and spleen d. Cyclins and caspases

Which one of the following cytokines is required very early in the differentiation of a hematopoietic stem cell? a. IL-2 b. IL-8 c. EPO d. FLT3 ligand

Physiologic programmed cell death is termed: a. Angiogenesis c. Aneurysm b. Apoptosis d. Apohematics

Erythropoietin can increase the production of RBCS by: a. Promoting apoptosis of erythroid progenitors b. Decreasing intravascular hemolysis c. Increasing EPO receptor sites d. Promoting early release of reticulocytes from bone marrow

What is the preferred bone marrow collection site in adults? a. Second intercostal space on the sternum b. Anterior or posterior iliac crest c. Any of the thoracic vertebrae d. Anterior head of the femur

What is the normal myeloid to erythroid (M:E) ratio range in adults? a. 1.5:1 to 3.3:1 b. 5.1:1 to 6.2:1 c. 8.6:1 to 10.2:1 d. 10:1 to 12:1

The aspirate should be examined under low power to assess all of the following except: a. Cellularity b. Megakaryocyte numbers c. Morphology of abnormal cells d. Presence of tumor cell clusters

Apoptosis is morphologically identified by: a. Cellular swelling b. Nuclear condensation. c. Rupture of the cytoplasm d. Rupture of the nucleus

When a patient has severe anemia and the bone marrow is unable to effectively produce red blood cells to meet the increased demand, one of the body’s responses is: a. Extramedullary hematopoiesis in the liver and spleen b. Decreased production of erythropoietin by the kidney c. Increased apoptosis of erythrocyte progenitor cells d. Increased proportion of yellow marrow in the long bones

During the second trimester of fetal development, the primary site of blood cell production is the: a. Bone marrow b. Spleen c. Lymph nodes d. Liver

Which of the following is not an indication for a bone marrow examination? a. Pancytopenia (reduced numbers of red blood cells, white blood cells, and platelets in the peripheral blood) b. Anemia with RBC indices corresponding to low serum iron and low ferritin levels c. Detection of blasts in the peripheral blood d. Need for staging of Hodgkin lymphoma

Which one of the following morphologic changes occurs during normal blood cell maturation? a. Increase in cell diameter b. Development of cytoplasm basophilia c. Condensation of nuclear chromatin d. Appearance of nucleol

What is the largest hematopoietic cell found in a normal bone marrow aspirate? a. Osteoblast b. Myeloblast c. Pronormoblast d. Megakaryocyte

Which organ is the site of sequestration of platelets? a. Liver b. Thymus c. Spleen d. Bone marrow

In the bone marrow, erythroid precursors are located: a. Surrounding macrophages in erythroid islands b. Adjacent to megakaryocytes along the adventitial cell c. Surrounding fat cells in apoptotic islands d. In the center of the hematopoietic cords

At which normoblastic stage does globin production begin? a. Orthochromic normoblast b. Pronormoblast. c. Polychromatic normoblast d. Basophilic normoblast

Which are the most common erythroid stages found in normal marrow? a. Pronormoblasts b. Pronormoblasts and basophilic normoblasts c. Basophilic and polychromatophilic normoblasts d. Polychromatophilic and orthochromic normoblasts

Which RBC process does not require energy? a. Cytoskeletal protein deformability b. Maintaining cytoplasm cationic electrochemical gradients c. Oxygen transport d. Preventing the peroxidation of proteins and lipids

What pathway anaerobically generates energy in the form of ATP? a. 2,3-BPG pathway b. Embden-Meyerhof pathway c. Hexose monophosphate pathway d. Rapoport-Luebering pathway

What hexose-monophosphate shunt products participate in the detoxification of peroxides? a. 2,3-BPG and pyruvic acid b. ATP and lactic acid c. NADPH and reduced glutathione d. Pyruvic and lactic acid

Hemoglobin iron may become oxidized to Fe3+ by several pathologic mechanisms. What portion of the EmbdenMeyerhof pathway reduces iron to Fe2+? a. Hexose monophosphate pathway b. Methemoglobin reductase pathway c. Rapoport-Luebering pathway d. 2,3-BPG shunt

Abnormalities in the horizontal and vertical linkages of the transmembrane and cytoskeletal RBC membrane proteins may be seen as: a. Enzyme pathway deficiencies b. Methemoglobin increase c. Reduced hemoglobin content d. Shape changes

A hemoglobin molecule is composed of: a. One heme molecule and four globin chains b. Ferrous iron, protoporphyrin IX, and a globin chain c. Protoporphyrin IX and four globin chains d. Four heme molecules and four globin chains

A key rate-limiting step in heme synthesis is suppression of: a. Aminolevulinate synthase b. Carbonic anhydrase c. Protoporphyrin IX reductase d. Glucose-6-phosphate dehydrogenase

Normal adult Hb A contains which polypeptide chains? a. alpha and beta c. alpha and gamma b. alpha and delta d. alpha and epsilon

How are the globin chain genes arranged? a. With a genes and b genes on the same chromosome, including two a genes and two b genes b. With a genes and b genes on separate chromosomes, including two a genes on one chromosome and one b gene on a different chromosome c. With a genes and b genes on the same chromosome, including four a genes and four b genes d. With a genes and b genes on separate chromosomes, including four a genes on one chromosome and two b genes on a different chromosome

What is the normal distribution of hemoglobins in healthy adults? a. 80% to 90% HbA, 5% to 10% HbA2 ,1% to 5% HbF b. 80% to 90% HbA2 ,5% to 10% HbA,1% to 5% HbF c. 95% HbA, 3.5% HbA2, 1% to 2% HbF d. 90% Hb A, 5% Hb F, 5% Hb A2

The predominant hemoglobin found in a healthy newborn a. Gower-1 b . Gower-2 c. A d. F

Which of the following forms of hemoglobin molecule has the lowest affinity for oxygen? a. Tense b. Relaxed

Using the normal hemoglobin-oxygen dissociation curve for reference, predict the position of the curve when there is a decrease in pH. a. Shifted to the right of normal with decreased oxygen affinity b. Shifted to the left of normal with increased oxygen affinity c. Shifted to the right of normal with increased oxygen affinity d. Shifted to the left of normal with decreased oxygen affinity

Which of the following is a description of the structure of oxidized hemoglobin? a. Hemoglobin carrying oxygen on heme; synonymous with oxygenated hemoglobin b. Hemoglobin with iron in the ferric state (methemoglobin) and not able to carry oxygen c. Hemoglobin with iron in the ferric state so that carbon dioxide replaces oxygen in the heme structure d. Hemoglobin carrying carbon monoxide; hence oxidized refers to the single oxygen

Following are several of the many steps in the process from absorption and transport of iron to incorporation into heme. Place them in proper order. i. Transferrin picks up ferric iron. ii. Iron is transferred to the mitochondria. iii. DMT1 transports ferrous iron into the enterocyte. iv. Ferroportin transports iron from enterocyte to plasma. v. The transferrin receptor transports iron into the cell. a. v,iv,i,ii,iii b. iii,ii,iv,i,v c. ii,i,v,iii,iv d. iii,iv,i,v,i

What erythroid precursor can be described as follows: The cell is of medium size compared with other normoblasts, with an N:C ratio of nearly 1:1. The nuclear chromatin is condensed and chunky throughout the nucleus. No nucleoli are seen. The cytoplasm is gray-blue. a. Reticulocyte b. Pronormoblast. c. Orthochromic normoblast d. Polychromatic normoblast

What membrane-associated protein in enterocytes transports iron from the intestinal lumen into the enterocyte? a. DMT1 b. Ferroportin c. Transferrin d. Hephaestin

Iron is transported out of macrophages, hepatocytes, and enterocytes by what membrane protein? a. Transferrin c. DMT1 b. Ferroportin d. Ferrochelatase

Which of the following cells critical to iron trafficking in the body does NOT possess ferroportin? a. Erythroblasts c. Hepatocytes b. Enterocytes d. Macrophages

The transfer of iron from the enterocyte into the plasma is REGULATED by: a. Transferrin c. Hephaestin b. Ferroportin d. Hepcidin

Developing erythroblasts in the bone marrow affect the supply of body iron, both absorption and recycling, by secreting which of the following hormones? a. Erythropoietin c. Hepcidin b. Erythroferrone d. Haptoglobin

What is the major metabolically available storage form of iron in the body? a. Hemosiderin c. Transferrin b. Ferritin d. Hemoglobin

Iron is transported in plasma via: a. Hemosiderin c. Transferrin b. Ferritin d. Hemoglobin

The total iron-binding capacity (TIBC) of the serum is an indirect measure of which iron-related protein? a. Hemosiderin c. Transferrin b. Ferritin d. Haptoglobin

For a patient with screening iron study values that are equivocal for iron deficiency, which of the following tests would be most helpful in determining whether iron deficiency is present or not? a. Zinc protoporphyrin b. Peripheral blood iron stain c. Soluble transferrin receptor d. Mean cell hemoglobin

What is the percent transferrin saturation for a patient with total serum iron of 63 mg/dL and TIBC of 420 mg/dL? a. 6.7% c. 15% b. 12% d. 80%

A physician is concerned that a patient is developing iron deficiency from chronic intestinal bleeding caused by aspirin use for rheumatoid arthritis. The iron studies on the patient indicate the following results: How would these results be interpreted? a. Latent iron deficiency b. Functional iron deficiency c. Iron deficiency d. Equivocal for iron deficiency

What are the initial laboratory tests that are performed for the diagnosis of anemia? a. CBC, iron studies, and reticulocyte count b. CBC, reticulocyte count, and peripheral blood film examination c. Reticulocyte count and serum iron, vitamin B12, and folate assays d. Bone marrow study, iron studies, and peripheral blood film examination

An increase in which one of the following suggests a shortened life span of RBCs and hemolytic anemia? a. Hemoglobin concentration c. Reticulocyte count b. Hematocrit d. Red cell distribution width

Which of the following is detectable only by examination of a peripheral blood film? a. Microcytosis. b. Anisocytosis c. Hypochromia d. Poikilocytosis

Determine which one of the following conditions would be included in the differential diagnosis of an anemic adult patient with an absolute reticulocyte count of 20 x 10^9/L and an MCV of 65 fL. a. Aplastic anemia b. Sickle cell anemia c. Iron deficiency d. Folate deficiency

Which one of the following conditions would be included in the differential diagnosis of an anemic adult patient with an MCV of 125 fL and an RDW of 20% a. Aplastic anemia c. Iron deficiency b. Sickle cell anemia d. Vitamin B12 deficiency

Predict the iron study results for the patient with Hodgkin lymphoma

In the pathogenesis of the anemia of chronic inflammation, hepcidin levels: a. Decrease during inflammation and reduce iron absorption from enterocytes b. Increase during inflammation and reduce iron absorption from enterocytes c. Increase during inflammation and increase iron absorption from enterocytes d. Decrease during inflammation and increase iron absorption from enterocytes

A 35-year-old white woman went to her physician complaining of headaches, dizziness, and nausea. The headaches had been increasing in severity over the past 6 months. This was coincident with her move into an older house built about 1900. She had been renovating the house, including stripping paint from the woodwork. Her CBC results showed a mild hypochromic, microcytic anemia, with polychromasia and basophilic stippling noted. Which of the following tests would be most useful in confirming the cause of her anemia? a. Serum lead level b. Serum iron level and TIBC c. Absolute reticulocyte count d. Prussian blue staining of the bone marrow to detect iron stores in macrophages

In what situation will increased levels of free erythrocyte protoporphyrin be present? a. Loss of function mutation to one of the enzymes in the heme synthesis pathway b. A mutation that prevents heme attachment to globin so that protoporphyrin remains free c. Any condition that prevents iron incorporation into protoporphyrin IX d. When red blood cells lyse, freeing their contents into the plasma

Folate and vitamin B12 work together in the production of: a. Amino acids. c. Phospholipids b. RNA d. DNA

Sideroblastic anemias result from: a. Sequestration of iron in hepatocytes b. Inability to incorporate heme into hemoglobin c. Sequestration of iron in myeloblasts d. Failure to incorporate iron into protoporphyrin IX

Which of the following findings is consistent with a diagnosis of megaloblastic anemia? a. Hyposegmentation of neutrophils b. Decreased serum lactate dehydrogenase level c. Absolute increase in reticulocytes d. Increased MCV

Which one of the following findings would be inconsistent with elevated titers of intrinsic factor blocking antibodies? a. Hypersegmentation of neutrophils b. Low levels of methylmalonic acid c. Macrocytic RBCs d. Low levels of vitamin B12

Idiopathic acquired aplastic anemia is due to a(n): a. Drug reaction c. Inherited mutation in stem cells b. Benzene exposure d. Unknown cause

The pathophysiologic mechanism in acquired idiosyncratic aplastic anemia is: a. Replacement of bone marrow cells by abnormal cells b. Destruction of stem cells by autoimmune T cells c. Defective production of hematopoietic growth factors d. Inability of bone marrow stroma to support stem cells

Diamond-Blackfan anemia differs from Fanconi anemia in that in the former: a. Reticulocyte count is increased b. Fetal hemoglobin is decreased c. Only erythropoiesis is affected d. Congenital malformations are absent

Which anemia should be suspected in a patient with i4nflammatory anemia, reticulocytopenia, hemosiderosis, and binucleated erythrocyte precursors in the bone marrow? a. Fanconi anemia b. Dyskeratosis congenita c. Acquired aplastic anemia d. Congenital dyserythropoietic anemia

The primary pathophysiologic mechanism of anemia associated with chronic kidney disease is: a. Inadequate production of erythropoietin b. Excessive hemolysis c. Hematopoietic stem cell mutation d. Toxic destruction of stem cells

Which one of the following findings is not consistent with myelophthisic anemia? a. Reticulocytosis c. Extramedullary hematopoiesis b. Teardrop RBCs d. Leukoerythroblastic blood picture

The term hemolytic disorder in general refers to a disorder in which there is: a. Increased destruction of RBCs in the blood, bone marrow, or spleen b. Excessive loss of RBCs from the body c. Inadequate RBC production by the bone marrow d. Increased plasma volume with unchanged red cell mass

RBC destruction that occurs when macrophages ingest and destroy RBCs is termed: a. Extracellular c. Intra-organ b. Macrophage mediated d. Extrahematopoietic

. A sign of hemolysis that is typically associated with both fragmentation and macrophage-mediated hemolysis is: a. Hemoglobinuria c. Hemoglobinemia b. Hemosiderinuria. d. Elevated urinary urobilinogen level

Which of the following tests provides a good indication of accelerated erythropoiesis? a. Urine urobilinogen level b. Hemosiderin level. c. Reticulocyte count d. Glycated hemoglobin level

A patient has a personal and family history of a mild hemolytic anemia. The patient has consistently elevated levels of total and indirect serum bilirubin and urinary urobilinogen. The serum haptoglobin level is consistently decreased, whereas the reticulocyte count is elevated. The latter can be seen as polychromasia on the patient’s peripheral blood film. Spherocytes are also noted. Which one of the findings reported for this patient is inconsistent with a classical diagnosis of fragmentation hemolysis? a. Elevated total and indirect serum bilirubin b. Elevated urinary urobilinogen c. Decreased haptoglobin d. Spherocytes on the peripheral blood film

Which of the following sets of test results is typically expected with chronic fragmentation hemolysis?

In HS, a characteristic abnormality in the CBC results a. Decreased MCH b. Decreased platelet and WBC counts c. Increased MCHC d. Increased MCV

n HS, a characteristic abnormality in the CBC results a. Decreased MCH b. Decreased platelet and WBC counts c. Increased MCHC d. Increased MCV

The altered shape of the spherocyte in HS is due to: a. Abnormal precipitation of the hemoglobin molecule b. A mutated RBC membrane protein affecting vertical protein interactions c. A mutated RBC membrane protein affecting horizontal protein interactions d. Defective RNA catabolism and clearance

The RBCs in HE are abnormally shaped and have unstable cell membranes as a result of: a. Defects in horizontal membrane protein interactions b. Deficiency in cation pumps in the RBC membrane c. Lack of Rh antigens in the RBC membrane d. Mutations in the ankyrin complex

Acanthocytes are found in association with: a. Abetalipoproteinemia c. Rh deficiency syndrome b. G6PD deficiency. d. Vitamin B12 deficiency

The most common defect or deficiency in the anaerobic glycolytic pathway that causes chronic HNSHA is: a. Glucose-6-phosphate dehydrogenase deficiency b. Lactate dehydrogenase deficiency c. Methemoglobin reductase deficiency d. Pyruvate kinase deficiency

The most common manifestation of G6PD deficiency a. Acute hemolytic anemia caused by drug exposure or infections b. Chronic hemolytic anemia caused by cell shape change c. Chronic hemolytic anemia caused by intravascular RBC lysis d. Mild compensated hemolysis caused by ATP deficiency

Which of the following laboratory tests would be best to confirm PNH? a. Acidified serum test (Ham test) b. Flow cytometry for detection of eosin-5’-maleimide binding on erythrocytes c. Flow cytometry for FLAER binding, CD24 on granulocytes, and CD14 on monocytes d. Osmotic fragility test

A 22-year-old man with a moderate decrease in hemoglobin level and a decrease in RBC, WBC, platelet, and reticulocyte counts has a history of infrequent and mild episodes of hemolysis with hemoglobinuria. His bone marrow showed 15% cellularity with no abnormal cells, and flow cytometry revealed that 15% of his circulating granulocytes were GPI deficient. He most likely has: a. A hereditary RBC membrane defect b. Classic PNH c. Hypoplastic PNH d. Subclinical PNH

What RBC morphology is characteristically found within the first 24 hours following extensive burn injury? a. Macrocytosis and polychromasia b. Burr cells and crenated cells c. Howell-Jolly bodies and bite cells d. Schistocytes and microspherocytes

Which one of the following is a feature found in all microangiopathic hemolytic anemias? a. Pancytopenia b. Thrombocytosis c. Intravascular RBC fragmentation d. Prolonged prothrombin time, partial thromboplastin time

Typical laboratory findings in TTP and HUS a. Schistocytosis and thrombocytopenia b. Anemia and reticulocytopenia c. Reduced levels of lactate dehydrogenase and aspartate aminotransferase d. Increased levels of free plasma hemoglobin and serum haptoglobin

The pathophysiology of idiopathic TTP involves: a. Shiga toxin damage to endothelial cells and obstruction of small blood vessels in glomeruli b. Formation of platelet-VWF thrombi due to autoantibody inhibition of ADAMTS13 c. Overactivation of the complement system and endothelial cell damage due to loss of regulatory function d. Activation of the coagulation and fibrinolytic systems with fibrin clots throughout the microvasculature

Which of the following tests yields results that are abnormal in DIC but are usually within the reference interval or just slightly abnormal in TTP and HUS? a. Indirect serum bilirubin and serum haptoglobin b. Prothrombin time and partial thromboplastin time c. Lactate dehydrogenase and aspartate aminotransferase d. Serum creatinine and serum total protein

A pregnant woman was seen in the emergency department with the following: decreased platelet count, proteinuria, fragmented RBCs, and increased liver enzymes. What is the most likely cause of these abnormalities? a. HELLP syndrome c. Antip

In hemolytic anemia, a positive DAT is evidence that a. IgM antibody is in the patient’s serum b. IgG antibody is in the patient’s serum c. IgM antibody is sensitizing the patient’s red blood cells d. IgG antibody is sensitizing the patient’s red blood cells

In hemolysis mediated by IgG antibodies, which abnormal RBC morphology is typically observed on the peripheral blood film? a. Spherocytes c. RBC agglutination b. Nucleated RBCs d. Macrocytes

Patients with SCD usually do not exhibit symptoms until 6 months of age because: a. The mother’s blood has a protective effect b. Hemoglobin levels are higher in infants at birth c. Higher levels of Hb F are present d. The immune system is not fully developed

The screening test for Hb S that uses a reducing agent, such as sodium dithionite, is based on the fact that hemoglobins that sickle: a. Are insoluble in reduced, deoxygenated form b. Form methemoglobin more readily and cause a color change c. Are unstable and precipitate as Heinz bodies d. Oxidize quickly and cause turbidity

Which of the following is the definitive test for Hb S? a. Hemoglobin solubility test b. Hemoglobin electrophoresis at alkaline pH c. Osmotic fragility test d. Hemoglobin electrophoresis at acid pH

A patient presents with mild normochromic, normocytic anemia. On the peripheral blood film, there are a few target cells, rare nucleated RBCs, and hexagonal crystals within and lying outside of the RBCs. Which abnormality in the hemoglobin molecule is most likely? a. Decreased production of b chains b. Substitution of lysine for glutamic acid at position 6 of the b chain c. Substitution of tyrosine for the proximal histidine in the b chain d. Double amino acid substitution in the b chain

A well-mixed specimen obtained for a CBC has a brown color. The patient is being treated with a sulfonamide for a bladder infection. Which of the following could explain the brown color? a. The patient has Hb M. b. The patient is a compound heterozygote for Hb S and thalassemia. c. The incorrect anticoagulant was used. d. Levels of Hb F are high.

Which hemoglobinopathy is more common in Southeast Asian patients? a. Hb S b. Hb C c. Hb O d. Hb E

A 1-year-old Indian patient presents with anemia, and both parents claim to have an “inherited anemia” but can’t remember the type. The peripheral blood shows target cells, and the hemoglobin solubility is negative. Alkaline hemoglobin electrophoresis shows a single band at the “Hb C” position and a small band at the “Hb F” position. Acid hemoglobin electrophoresis shows two bands. The most likely diagnosis is: a. Hb CC c. Hb CO b. HbAC d. Hb SC

The hemolytic anemia and ineffective erythropoiesis associated with Beta-thalassemia is due to: a. A structurally abnormal hemoglobin b. Oxidation of hemoglobin to Heinz bodies c. Uncoupling of the RBC membrane from the cyto-skeleton d. Precipitation of excess a chains in RBCs and their precursors

Thalassemia is caused by: a. Structurally abnormal hemoglobins b. Absent or reduced synthesis of a polypeptide chain of hemoglobin c. Excessive absorption of iron d. Reduced or absent protoporphyrin synthesis

Beta-thalassemia minor (heterozygous) usually exhibits: a. Increased Hb H c. No Hb A b. 10% to 35% HbF d. Increased Hb A2

Which of the following laboratory findings is inconsistent with Beta thalassemia minor? a. A slightly elevated RBC count and marked microcytosis b. Target cells and basophilic stippling on the peripheral blood film c. Hemoglobin level of 10 to 13 g/dL d. Elevated MCHC and spherocytic RBCs

When one alpha gene is deleted (a–/aa), a patient has: a. Normal hemoglobin levels b. Mild anemia (hemoglobin range 9 to 11 g/dL) c. Moderate anemia (hemoglobin range 7 to 9 g/dL) d. Marked anemia requiring regular transfusions

What abnormal hemoglobin is present in adults with alpha thalassemia, genotype (– –/a –)? a. A2 b. F c. H d. Bart